Updated on 2022/06/11

写真a

 
KATSUNO, Masahisa
 
Organization
Graduate School of Medicine Program in Integrated Medicine Clinical Neurosciences Professor
Graduate School
Graduate School of Medicine
Undergraduate School
School of Medicine Department of Medicine
Title
Professor

Degree 1

  1. Doctor of Medicine ( 2003.3   Nagoya University ) 

Research Interests 5

  1. clinical trial

  2. translational research

  3. spinal and bulbar muscular atrrophy

  4. polyglutamine disease

  5. neurodegenerative disease

Research Areas 4

  1. Others / Others  / Neurology

  2. Others / Others  / Cell Biology

  3. Others / Others  / 神経科学

  4. Others / Others  / 臨床試験

Research History 9

  1. Nagoya University   Department of Neurology, Graduate School of Medicine   Professor

    2015.7

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    Country:Japan

  2. Nagoya University   Department of Neurology, Graduate School of Medicine   Associate professor

    2012.12 - 2015.6

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    Country:Japan

  3. Nagoya University   Department of Neurology, Graduate School of Medicine   Designated associate professor

    2011.4 - 2012.11

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    Country:Japan

  4. Associate Professor, Institute for Advanced Research, Nagoya University

    2007.11 - 2011.3

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    Country:Japan

  5. Research Resident, Japan Foundation for Aging and Health

    2005.10 - 2006.10

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    Country:Japan

  6. Reserch Resident, Japan Foundation for Aging and Health

    2004.4 - 2005.3

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    Country:Japan

  7. Research Fellow, Nagoya University School of Medicine, Nagoya, Japan

    2004.4 - 2004.9

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    Country:Japan

  8. Department of Neurology, Nagoya Daini Red Cross Hospital

    1997.4 - 2000.3

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    Country:Japan

  9. Resident, Nagoya Daini Red Cross Hospital

    1995.4 - 1997.5

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    Country:Japan

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Education 2

  1. Nagoya University   Graduate School, Division of Medical Sciences

    2000.4 - 2003.3

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    Country: Japan

  2. Nagoya University   Faculty of Medicine

    1989.4 - 1995.3

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    Country: Japan

Professional Memberships 10

  1. 日本神経学会   理事

    2018.5

  2. 日本臨床薬理学会   地方会世話人

    2014.12

  3. 日本神経治療学会   評議員

    2011.12

  4. 日本認知症学会

  5. 日本神経科学会

  6. 日本末梢神経学会

  7. 日本自律神経学会

  8. 日本健診医学会

  9. Society for Neuroscience

  10. 日本内科学会

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Awards 3

  1. Commendation for Science and Technology by the Minister of Education, Culture, Sports, Science and Technology

    2020.4   MEXT, Japan  

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    Country:Japan

  2. 日本神経学会賞

    2014.5   日本神経学会  

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    Award type:International academic award (Japan or overseas)  Country:Japan

  3. 日本学術振興会賞

    2009.3   日本学術振興会  

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    Country:Japan

 

Papers 368

  1. Short chain fatty acids-producing and mucin-degrading intestinal bacteria predict the progression of early Parkinson's disease.

    Nishiwaki H, Ito M, Hamaguchi T, Maeda T, Kashihara K, Tsuboi Y, Ueyama J, Yoshida T, Hanada H, Takeuchi I, Katsuno M, Hirayama M, Ohno K

    NPJ Parkinson's disease   Vol. 8 ( 1 ) page: 65   2022.6

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    Language:English  

    DOI: 10.1038/s41531-022-00328-5

    PubMed

  2. [Recommendations (Proposal) for promoting research for overcoming neurological diseases 2020].

    Mochizuki H, Aoki M, Ikenaka K, Inoue H, Iwatsubo T, Ugawa Y, Okazawa H, Ono K, Onodera O, Kitagawa K, Saito Y, Shimohata T, Takahashi R, Toda T, Nakahara J, Matsumoto R, Mizusawa H, Mitsui J, Murayama S, Katsuno M, Future Vision Committee of Japanese Society of Neurology., Aoki Y, Ishiura H, Izumi Y, Koike H, Shimada H, Takahashi Y, Tokuda T, Nakajima H, Hatano T, Misawa S, Watanabe H

    Rinsho shinkeigaku = Clinical neurology     2022.5

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    Language:Japanese  

    DOI: 10.5692/clinicalneurol.cn-001695

    PubMed

  3. [Recommendations (Proposal) for promoting research for overcoming neurological diseases 2020].

    Mochizuki H, Aoki M, Ikenaka K, Inoue H, Iwatsubo T, Ugawa Y, Okazawa H, Ono K, Onodera O, Kitagawa K, Saito Y, Shimohata T, Takahashi R, Toda T, Nakahara J, Matsumoto R, Mizusawa H, Mitsui J, Murayama S, Katsuno M, Future Vision Committee of Japanese Society of Neurology., Aoki Y, Ishiura H, Izumi Y, Koike H, Shimada H, Takahashi Y, Tokuda T, Nakajima H, Hatano T, Misawa S, Watanabe H

    Rinsho shinkeigaku = Clinical neurology     2022.5

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    Language:Japanese  

    DOI: 10.5692/clinicalneurol.cn-001696

    PubMed

  4. Actin-binding protein filamin-A drives tau aggregation and contributes to progressive supranuclear palsy pathology.

    Tsujikawa K, Hamanaka K, Riku Y, Hattori Y, Hara N, Iguchi Y, Ishigaki S, Hashizume A, Miyatake S, Mitsuhashi S, Miyazaki Y, Kataoka M, Jiayi L, Yasui K, Kuru S, Koike H, Kobayashi K, Sahara N, Ozaki N, Yoshida M, Kakita A, Saito Y, Iwasaki Y, Miyashita A, Iwatsubo T, Japanese Alzheimer’s Disease Neuroimaging Initiative (J-ADNI)., Ikeuchi T, Japanese Longitudinal Biomarker Study in PSP and CBD (JALPAC) Consortium., Miyata T, Sobue G, Matsumoto N, Sahashi K, Katsuno M

    Science advances   Vol. 8 ( 21 ) page: eabm5029   2022.5

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    DOI: 10.1126/sciadv.abm5029

    PubMed

  5. Neurolymphomatosis in follicular lymphoma: an autopsy case report

    Ando Takashi, Kamoshita Sonoko, Riku Yuichi, Ito Ai, Ozawa Yukiyasu, Miyamura Koichi, Fujino Masahiko, Ito Masafumi, Goto Yoji, Mano Kazuo, Akagi Akio, Miyahara Hiroaki, Katsuno Masahisa, Yoshida Mari, Iwasaki Yasushi

    NEUROPATHOLOGY     2022.5

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  6. Role of complement components in vasculitic neuropathy associated with systemic lupus erythematosus and rheumatoid arthritis.

    Fukami Y, Koike H, Iijima M, Mouri N, Nishi R, Katsuno M

    Muscle & nerve     2022.5

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    DOI: 10.1002/mus.27636

    PubMed

  7. Novel approach to semi-quantification of tracer accumulation in dopamine transporter scan

    Ito Yoshinori, Fujita Naotoshi, Hara Kazuhiro, Tada Tomohiro, Abe Shinji, Katsuno Masahisa, Naganawa Shinji, Kato Katsuhiko

    JOURNAL OF APPLIED CLINICAL MEDICAL PHYSICS     page: e13626   2022.5

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  8. Safety and Clinical Benefits of Laryngeal Closure in Patients with Amyotrophic Lateral Sclerosis

    Yokoi Sayaka, Nishio Naoki, Maruo Takashi, Hiramatsu Mariko, Mukoyama Nobuaki, Tsuzuki Hidenori, Wada Akihisa, Atsuta Naoki, Ito Daisuke, Tsuboi Takashi, Sobue Gen, Katsuno Masahisa, Fujimoto Yasushi, Sone Michihiko

    DYSPHAGIA     2022.5

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  9. Mutation screening of the DNAJC7 gene in Japanese patients with sporadic amyotrophic lateral sclerosis.

    Tohnai G, Nakamura R, Atsuta N, Nakatochi M, Hayashi N, Ito D, Watanabe H, Watanabe H, Katsuno M, Izumi Y, Taniguchi A, Kanai K, Morita M, Kano O, Kuwabara S, Oda M, Abe K, Aoki M, Aiba I, Okamoto K, Mizoguchi K, Ishihara T, Kawata A, Yokota T, Hasegawa K, Nagano I, Yabe I, Tanaka F, Kuru S, Hattori N, Nakashima K, Kaji R, Sobue G, Japanese Consortium for Amyotrophic Lateral Sclerosis Research (JaCALS).

    Neurobiology of aging   Vol. 113   page: 131 - 136   2022.5

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  10. Serum asymmetric dimethylarginine level correlates with the progression and prognosis of amyotrophic lateral sclerosis.

    Ikenaka K, Maeda Y, Hotta Y, Nagano S, Yamada S, Ito D, Torii R, Kakuda K, Tatebe H, Atsuta N, Aguirre C, Kimura Y, Baba K, Tokuda T, Katsuno M, Kimura K, Sobue G, Mochizuki H

    European journal of neurology   Vol. 29 ( 5 ) page: 1410 - 1416   2022.5

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    DOI: 10.1111/ene.15254

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  11. Novel concept to evaluate efficacy of therapeutics for ALS based on patient preference comment

    Hashizume Atsushi, Katsuno Masahisa

    JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY   Vol. 93 ( 5 ) page: 457 - 457   2022.5

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  12. Functional connector hubs in the cerebellum.

    Kawabata K, Bagarinao E, Watanabe H, Maesawa S, Mori D, Hara K, Ohdake R, Masuda M, Ogura A, Kato T, Koyama S, Katsuno M, Wakabayashi T, Kuzuya M, Hoshiyama M, Isoda H, Naganawa S, Ozaki N, Sobue G

    NeuroImage   Vol. 257   page: 119263   2022.4

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    DOI: 10.1016/j.neuroimage.2022.119263

    PubMed

  13. In vivo visualization of eosinophil secretion in eosinophilic granulomatosis with polyangiitis: An ultrastructural study.

    Koike H, Nishi R, Furukawa S, Mouri N, Fukami Y, Iijima M, Katsuno M

    Allergology international : official journal of the Japanese Society of Allergology     2022.4

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    DOI: 10.1016/j.alit.2022.02.009

    PubMed

  14. Clinical diagnosis sensitivity of neuropathologically established multiple system atrophy in Japan

    Ando Takashi, Riku Yuichi, Akagi Akio, Miyahara Hiroaki, Sone Jun, Katsuno Masahisa, Yoshida Mari, Iwasaki Yasushi

    JOURNAL OF NEUROLOGY     2022.4

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  15. Dosage effects of PMP22 on nonmyelinating Schwann cells in hereditary neuropathy with liability to pressure palsies.

    Koike H, Furukawa S, Mouri N, Fukami Y, Iijima M, Katsuno M

    Neuromuscular disorders : NMD     2022.4

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    DOI: 10.1016/j.nmd.2022.04.002

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  16. Quantitative evaluation of upper limb ataxia in spinocerebellar ataxias

    Kishimoto Yoshiyuki, Hashizume Atsushi, Imai Yuta, Nakatochi Masahiro, Yamada Shinichiro, Ito Daisuke, Torii Ryota, Nagano Yoshitaka, Fujimoto Hideo, Katsuno Masahisa

    ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY   Vol. 9 ( 4 ) page: 529 - 539   2022.4

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  17. Status Cataplecticus with Rapid Eye Movement Sleep Excess in Late-onset Narcolepsy Type 1.

    Ando T, Wakai M, Kanbayashi T, Katsuno M

    Internal medicine (Tokyo, Japan)     2022.3

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    DOI: 10.2169/internalmedicine.8465-21

    PubMed

  18. Motor neuron TDP-43 proteinopathy in progressive supranuclear palsy and corticobasal degeneration

    Riku Yuichi, Iwasaki Yasushi, Ishigaki Shinsuke, Akagi Akio, Hasegawa Masato, Nishioka Kenya, Li Yuanzhe, Riku Miho, Ikeuchi Takeshi, Fujioka Yusuke, Miyahara Hiroaki, Sone Jun, Hattori Nobutaka, Yoshida Mari, Katsuno Masahisa, Sobue Gen

    BRAIN     2022.3

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  19. ANCA-Associated Vasculitic Neuropathies: A Review

    Koike Haruki, Nishi Ryoji, Ohyama Ken, Morozumi Saori, Kawagashira Yuichi, Furukawa Soma, Mouri Naohiro, Fukami Yuki, Iijima Masahiro, Sobue Gen, Katsuno Masahisa

    NEUROLOGY AND THERAPY   Vol. 11 ( 1 ) page: 21 - 38   2022.3

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  20. Severe and long-lasting neuropsychiatric symptoms after mild respiratory symptoms caused by COVID-19: A case report.

    Jozuka R, Kimura H, Uematsu T, Fujigaki H, Yamamoto Y, Kobayashi M, Kawabata K, Koike H, Inada T, Saito K, Katsuno M, Ozaki N

    Neuropsychopharmacology reports   Vol. 42 ( 1 ) page: 114 - 119   2022.3

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    DOI: 10.1002/npr2.12222

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  21. Retinitis pigmentosa with optic neuropathy and <i>COQ2</i> mutations: A case report.

    Kurata K, Hosono K, Takayama M, Katsuno M, Saitsu H, Ogata T, Hotta Y

    American journal of ophthalmology case reports   Vol. 25   page: 101298   2022.3

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    DOI: 10.1016/j.ajoc.2022.101298

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  22. Randomized phase 2 study of perampanel for sporadic amyotrophic lateral sclerosis.

    Aizawa H, Kato H, Oba K, Kawahara T, Okubo Y, Saito T, Naito M, Urushitani M, Tamaoka A, Nakamagoe K, Ishii K, Kanda T, Katsuno M, Atsuta N, Maeda Y, Nagai M, Nishiyama K, Ishiura H, Toda T, Kawata A, Abe K, Yabe I, Takahashi-Iwata I, Sasaki H, Warita H, Aoki M, Sobue G, Mizusawa H, Matsuyama Y, Haga T, Kwak S

    Journal of neurology   Vol. 269 ( 2 ) page: 885 - 896   2022.2

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    DOI: 10.1007/s00415-021-10670-y

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  23. Fiber-specific white matter analysis reflects upper motor neuron impairment in amyotrophic lateral sclerosis

    Ogura Aya, Kawabata Kazuya, Watanabe Hirohisa, Choy Shao Wei, Bagarinao Epifanio, Kato Toshiyasu, Imai Kazunori, Masuda Michihito, Ohdake Reiko, Hara Kazuhiro, Nakamura Ryoichi, Atsuta Naoki, Nakamura Tomohiko, Katsuno Masahisa, Sobue Gen

    EUROPEAN JOURNAL OF NEUROLOGY   Vol. 29 ( 2 ) page: 432 - 440   2022.2

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  24. Unique Phenotypes With Corresponding Pathology in Late-Onset Hereditary Transthyretin Amyloidosis of A97S vs. V30M

    Hsueh Hsueh-Wen, Chao Chi-Chao, Chang Koping, Jeng Yung-Ming, Katsuno Masahisa, Koike Haruki, Hsieh Sung-Tsang

    FRONTIERS IN AGING NEUROSCIENCE   Vol. 13   page: 786322   2022.1

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  25. [What and How to Study in Graduate School].

    Katsuno M

    Brain and nerve = Shinkei kenkyu no shinpo   Vol. 74 ( 1 ) page: 25 - 28   2022.1

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    DOI: 10.11477/mf.1416201971

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  26. Nucleic Acid-Based Therapeutic Approach for Spinal and Bulbar Muscular Atrophy and Related Neurological Disorders

    Hirunagi Tomoki, Sahashi Kentaro, Meilleur Katherine G., Katsuno Masahisa

    GENES   Vol. 13 ( 1 )   2022.1

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  27. Multiple system atrophy variant with severe hippocampal pathology.

    Ando T, Riku Y, Akagi A, Miyahara H, Hirano M, Ikeda T, Yabata H, Koizumi R, Oba C, Morozumi S, Yasui K, Goto A, Katayama T, Sakakibara S, Aiba I, Sakai M, Konagaya M, Mori K, Ito Y, Yuasa H, Nomura M, Porto KJL, Mitsui J, Tsuji S, Mimuro M, Hashizume Y, Katsuno M, Iwasaki Y, Yoshida M

    Brain pathology (Zurich, Switzerland)   Vol. 32 ( 1 ) page: e13002   2022.1

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    DOI: 10.1111/bpa.13002

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  28. Reserve and Maintenance in the Aging Brain: A Longitudinal Study of Healthy Older Adults

    Bagarinao Epifanio, Watanabe Hirohisa, Maesawa Satoshi, Kawabata Kazuya, Hara Kazuhiro, Ohdake Reiko, Ogura Aya, Mori Daisuke, Yoneyama Noritaka, Imai Kazunori, Yokoi Takamasa, Kato Toshiyasu, Koyama Shuji, Katsuno Masahisa, Wakabayashi Toshihiko, Kuzuya Masafumi, Hoshiyama Minoru, Isoda Haruo, Naganawa Shinji, Ozaki Norio, Sobue Gen

    ENEURO   Vol. 9 ( 1 )   2022

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  29. Multidisciplinary Approaches for Transthyretin Amyloidosis

    Koike Haruki, Okumura Takahiro, Murohara Toyoaki, Katsuno Masahisa

    CARDIOLOGY AND THERAPY   Vol. 10 ( 2 ) page: 289 - 311   2021.12

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  30. Emerging Infection, Vaccination, and Guillain-Barre Syndrome: A Review

    Koike Haruki, Chiba Atsuro, Katsuno Masahisa

    NEUROLOGY AND THERAPY   Vol. 10 ( 2 ) page: 523 - 537   2021.12

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  31. Unexpected postmortem diagnoses in cases of clinically diagnosed amyotrophic lateral sclerosis

    Riku Yuichi, Yoshida Mari, Tamura Takuya, Kamijo Mikiko, Yasui Keizo, Kameyama Takashi, Katsuno Masahisa, Sobue Gen, Iwasaki Yasushi

    NEUROPATHOLOGY   Vol. 41 ( 6 ) page: 457 - 467   2021.12

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  32. [Recommendations (Proposal) for promoting research for overcoming neurological diseases 2020].

    Mochizuki H, Aoki M, Ikenaka K, Inoue H, Iwatsubo T, Ugawa Y, Okazawa H, Ono K, Onodera O, Kitagawa K, Saito Y, Shimohata T, Takahashi R, Toda T, Nakahara J, Matsumoto R, Mizusawa H, Mitsui J, Murayama S, Katsuno M, Future Vision Committee of Japanese Society of Neurology., Aoki Y, Ishiura H, Izumi Y, Koike H, Shimada H, Takahashi Y, Tokuda T, Nakajima H, Hatano T, Misawa S, Watanabe H

    Rinsho shinkeigaku = Clinical neurology   Vol. 61 ( 11 ) page: 709 - 721   2021.11

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    Language:Japanese  

    DOI: 10.5692/clinicalneurol.cn-001639

    PubMed

  33. Nemaline Myopathy Initially Diagnosed as Right Heart Failure with Type 2 Respiratory Failure.

    Ito M, Shima S, Ryunosuke N, Nakano S, Esaka K, Ueda A, Maeda S, Moriya R, Kondo M, Imaizumi K, Noda S, Katsuno M, Nishino I, Watanabe H

    Internal medicine (Tokyo, Japan)     2021.11

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    DOI: 10.2169/internalmedicine.8314-21

    PubMed

  34. Resting State Networks Related to the Maintenance of Good Cognitive Performance During Healthy Aging

    Maesawa Satoshi, Mizuno Satomi, Bagarinao Epifanio, Watanabe Hirohisa, Kawabata Kazuya, Hara Kazuhiro, Ohdake Reiko, Ogura Aya, Mori Daisuke, Nakatsubo Daisuke, Isoda Haruo, Hoshiyama Minoru, Katsuno Masahisa, Saito Ryuta, Ozaki Norio, Sobue Gen

    FRONTIERS IN HUMAN NEUROSCIENCE   Vol. 15   page: 753836   2021.11

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  35. Association between neurosarcoidosis with autonomic dysfunction and anti-ganglionic acetylcholine receptor antibodies.

    Oishi M, Mukaino A, Kunii M, Saito A, Arita Y, Koike H, Higuchi O, Maeda Y, Abiru N, Yamaguchi N, Kawano H, Tsuiki E, Tanaka T, Matsuo H, Katsuno M, Tanaka F, Tsujino A, Nakane S

    Journal of neurology   Vol. 268 ( 11 ) page: 4265 - 4279   2021.11

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  36. Instability of speech in Parkinson disease patients with subthalamic nucleus deep brain stimulation

    Tanaka Yasuhiro, Tsuboi Takashi, Watanabe Hirohisa, Torii Jun, Nakatsubo Daisuke, Maesawa Satoshi, Sato Maki, Hiraga Keita, Satake Yuki, Yokoi Katsunori, Hattori Makoto, Kawabata Kazuya, Hara Kazuhiro, Yamamoto Masahiko, Sobue Gen, Katsuno Masahisa

    PARKINSONISM & RELATED DISORDERS   Vol. 93   page: 8 - 11   2021.11

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  37. Paraproteinemia and neuropathy

    Koike Haruki, Katsuno Masahisa

    NEUROLOGICAL SCIENCES   Vol. 42 ( 11 ) page: 4489 - 4501   2021.11

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  38. Nerve biopsy in acquired neuropathies

    Sommer Claudia, Carroll Antonia S., Koike Haruki, Katsuno Masahisa, Ort Nora, Sobue Gen, Vucic Steve, Spies Judith M., Doppler Kathrin, Kiernan Matthew C.

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 26   page: S21 - S41   2021.11

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  39. Bridging large-scale cortical networks: Integrative and function-specific hubs in the thalamus

    Kawabata Kazuya, Bagarinao Epifanio, Watanabe Hirohisa, Maesawa Satoshi, Mori Daisuke, Hara Kazuhiro, Ohdake Reiko, Masuda Michihito, Ogura Aya, Kato Toshiyasu, Koyama Shuji, Katsuno Masahisa, Wakabayashi Toshihiko, Kuzuya Masafumi, Hoshiyama Minoru, Isoda Haruo, Naganawa Shinji, Ozaki Norio, Sobue Gen

    ISCIENCE   Vol. 24 ( 10 ) page: 103106   2021.10

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  40. Association of serum neurofilament light chain levels with clinicopathology of chronic inflammatory demyelinating polyneuropathy, including NF155 reactive patients

    Fukami Yuki, Iijima Masahiro, Koike Haruki, Yamada Shinichiro, Hashizume Atsushi, Katsuno Masahisa

    JOURNAL OF NEUROLOGY   Vol. 268 ( 10 ) page: 3835 - 3844   2021.10

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  41. Ratio of urinary N-terminal titin fragment to urinary creatinine is a novel biomarker for amyotrophic lateral sclerosis

    Yamada Shinichiro, Hashizume Atsushi, Hijikata Yasuhiro, Ito Daisuke, Kishimoto Yoshiyuki, Iida Madoka, Koike Haruki, Hirakawa Akihiro, Katsuno Masahisa

    JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY   Vol. 92 ( 10 ) page: 1072 - 1079   2021.10

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  42. The hot cross bun sign in corticobasal degeneration

    Ando Takashi, Yokoi Fuji, Riku Yuichi, Akagi Akio, Miyahara Hiroaki, Hasegawa Masato, Katsuno Masahisa, Yoshida Mari, Iwasaki Yasushi

    NEUROPATHOLOGY   Vol. 41 ( 5 ) page: 376 - 380   2021.10

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  43. Blockade of CHRNB2 signaling with a therapeutic monoclonal antibody attenuates the aggressiveness of gastric cancer cells

    Kanda Mitsuro, Shimizu Dai, Nakamura Shunsuke, Sawaki Koichi, Umeda Shinichi, Miwa Takashi, Tanaka Haruyoshi, Inokawa Yoshikuni, Hattori Norifumi, Hayashi Masamichi, Tanaka Chie, Nakayama Goro, Iguchi Yohei, Katsuno Masahisa, Kodera Yasuhiro

    ONCOGENE   Vol. 40 ( 36 ) page: 5495 - 5504   2021.9

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  44. A neuromuscular-based analysis of the open payments program (vol 63, pg 96, 2021)

    Harada Yohei, Sheng Sen, Thombre Vaishali A., Ounpraseuth Songthip, Tommee Carolina Gil, Annapureddy Amarnath R., Kamran Mudassar, Jasti Madhu S., Katsuno Masahisa, Yadala Sisira, Veerapaneni Karthika, Kapoor Nidhi, Kovvuru Sukanthi, Onteddu Sanjeeva Reddy, Nalleballe Krishna

    MUSCLE & NERVE   Vol. 64 ( 3 ) page: E17 - E17   2021.9

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    Language:Japanese  

    DOI: 10.1002/mus.27357

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  45. Individual voxel-based morphometry adjusting covariates in multiple system atrophy

    Ebina Junya, Hara Kazuhiro, Watanabe Hirohisa, Kawabata Kazuya, Yamashita Fumio, Kawaguchi Atsushi, Yoshida Yusuke, Kato Toshiyasu, Ogura Aya, Masuda Michihito, Ohdake Reiko, Mori Daisuke, Maesawa Satoshi, Katsuno Masahisa, Kano Osamu, Sobue Gen

    PARKINSONISM & RELATED DISORDERS   Vol. 90   page: 114 - 119   2021.9

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  46. Impaired pain processing and its association with attention disturbance in patients with amyotrophic lateral sclerosis

    Harada Yumiko, Nakamura Tomohiko, Suzuki Masashi, Ueda Masamichi, Hirayama Masaaki, Katsuno Masahisa

    NEUROLOGICAL SCIENCES   Vol. 42 ( 8 ) page: 3327 - 3335   2021.8

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  47. A case of sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance: long-term observation of neurological symptoms after autologous stem-cell transplantation

    Ando Takashi, Sato Takahiko, Kurahashi Shingo, Kawaguchi Yuka, Kagaya Yusuke, Ozawa Yukiyasu, Hirano Satoko, Goto Yoji, Mano Kazuo, Yokoi Satoshi, Nakamura Tomohiko, Murakami Ayuka, Noda Seiya, Kimura Seigo, Sone Jun, Kuru Satoshi, Sobue Gen, Katsuno Masahisa

    NAGOYA JOURNAL OF MEDICAL SCIENCE   Vol. 83 ( 3 ) page: 641 - 647   2021.8

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  48. The Ultrastructure of Tissue Damage by Amyloid Fibrils

    Koike Haruki, Katsuno Masahisa

    MOLECULES   Vol. 26 ( 15 )   2021.8

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  49. Significance of Oligomeric and Fibrillar Species in Amyloidosis: Insights into Pathophysiology and Treatment

    Koike Haruki, Iguchi Yohei, Sahashi Kentaro, Katsuno Masahisa

    MOLECULES   Vol. 26 ( 16 )   2021.8

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  50. DNA damage in embryonic neural stem cell determines FTLDs' fate via early-stage neuronal necrosis.

    Homma H, Tanaka H, Jin M, Jin X, Huang Y, Yoshioka Y, Bertens CJ, Tsumaki K, Kondo K, Shiwaku H, Tagawa K, Akatsu H, Atsuta N, Katsuno M, Furukawa K, Ishiki A, Waragai M, Ohtomo G, Iwata A, Yokota T, Inoue H, Arai H, Sobue G, Sone M, Fujita K, Okazawa H

    Life science alliance   Vol. 4 ( 7 )   2021.7

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    Language:English  

    DOI: 10.26508/lsa.202101022

    PubMed

  51. Selective suppression of polyglutamine-expanded protein by lipid nanoparticle-delivered siRNA targeting CAG expansions in the mouse CNS

    Hirunagi Tomoki, Sahashi Kentaro, Tachikawa Kiyoshi, Leu Angel I., Nguyen Michelle, Mukthavaram Rajesh, Karmali Priya P., Chivukula Padmanabh, Tohnai Genki, Iida Madoka, Onodera Kazunari, Ohyama Manabu, Okada Yohei, Okano Hideyuki, Katsuno Masahisa

    MOLECULAR THERAPY-NUCLEIC ACIDS   Vol. 24   page: 1 - 10   2021.6

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  52. Exploring the factors underlying remyelination arrest by studying the post-transcriptional regulatory mechanisms of cystatin F gene.

    Li J, Durose WW, Ito J, Kakita A, Iguchi Y, Katsuno M, Kunisawa K, Shimizu T, Ikenaka K

    Journal of neurochemistry   Vol. 157 ( 6 ) page: 2070 - 2090   2021.6

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    DOI: 10.1111/jnc.15190

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  53. Emerging infectious diseases, vaccines and Guillain-Barré syndrome.

    Koike H, Katsuno M

    Clinical & experimental neuroimmunology     2021.5

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    DOI: 10.1111/cen3.12644

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  54. The role of macrophages in Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy

    Koike Haruki, Katsuno Masahisa

    NEUROLOGY AND CLINICAL NEUROSCIENCE   Vol. 9 ( 3 ) page: 203 - 210   2021.5

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    DOI: 10.1111/ncn3.12496

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  55. Clinicoradiological features in amyotrophic lateral sclerosis patients with olfactory dysfunction

    Masuda Michihito, Watanabe Hirohisa, Ogura Aya, Ohdake Reiko, Kato Toshiyasu, Kawabata Kazuya, Hara Kazuhiro, Nakamura Ryoichi, Atsuta Naoki, Epifanio Bagarinao, Katsuno Masahisa, Sobue Gen

    AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION   Vol. 22 ( 3-4 ) page: 260 - 266   2021.4

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  56. The wide-ranging clinical and genetic features in Japanese families with valosin-containing protein proteinopathy.

    Ando T, Nakamura R, Kuru S, Yokoi D, Atsuta N, Koike H, Suzuki M, Hara K, Iguchi Y, Harada Y, Yoshida Y, Hattori M, Murakami A, Noda S, Kimura S, Sone J, Nakamura T, Goto Y, Mano K, Okada H, Okuda S, Nishino I, Ogi T, Sobue G, Katsuno M

    Neurobiology of aging   Vol. 100   page: 120.e1 - 120.e6   2021.4

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  57. ANCA-associated neuropathy in systemic sclerosis: A case report and review of literature

    Takenaka Kayo, Takeichi Takuya, Nishi Ryoji, Suzuki Masashi, Koike Haruki, Katsuno Masahisa, Ogawa-Momohara Mariko, Muro Yoshinao, Akiyama Masashi

    JOURNAL OF CUTANEOUS IMMUNOLOGY AND ALLERGY   Vol. 4 ( 2 ) page: 34 - 36   2021.4

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    DOI: 10.1002/cia2.12153

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  58. Macrophages and Autoantibodies in Demyelinating Diseases

    Koike Haruki, Katsuno Masahisa

    CELLS   Vol. 10 ( 4 )   2021.4

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  59. Pathway from TDP-43-Related Pathology to Neuronal Dysfunction in Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration

    Riku Yuichi, Seilhean Danielle, Duyckaerts Charles, Boluda Susana, Iguchi Yohei, Ishigaki Shinsuke, Iwasaki Yasushi, Yoshida Mari, Sobue Gen, Katsuno Masahisa

    INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES   Vol. 22 ( 8 )   2021.4

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  60. Efficacy and Safety of Bimagrumab in Sporadic Inclusion Body Myositis: Long-term Extension of RESILIENT.

    Amato AA, Hanna MG, Machado PM, Badrising UA, Chinoy H, Benveniste O, Karanam AK, Wu M, Tankó LB, Schubert-Tennigkeit AA, Papanicolaou DA, Lloyd TE, Needham M, Liang C, Reardon KA, de Visser M, Ascherman DP, Barohn RJ, Dimachkie MM, Miller JAL, Kissel JT, Oskarsson B, Joyce NC, Van den Bergh P, Baets J, De Bleecker JL, Karam C, David WS, Mirabella M, Nations SP, Jung HH, Pegoraro E, Maggi L, Rodolico C, Filosto M, Shaibani AI, Sivakumar K, Goyal NA, Mori-Yoshimura M, Yamashita S, Suzuki N, Aoki M, Katsuno M, Morihata H, Murata K, Nodera H, Nishino I, Romano CD, Williams VSL, Vissing J, Zhang Auberson L, RESILIENT Study Extension Group.

    Neurology   Vol. 96 ( 12 ) page: e1595 - e1607   2021.3

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  61. Case Report: Severe Osteoporosis and Preventive Therapy in RNA Polymerase III-Related Leukodystrophy

    Furukawa Soma, Kunii Misako, Doi Hiroshi, Kondo Naohide, Ogura Aya, Hirabuki Koichi, Itoh Takayuki, Matsumoto Naomichi, Tanaka Fumiaki, Katsuno Masahisa, Ito Yasuhiro

    FRONTIERS IN NEUROLOGY   Vol. 12   page: 622355   2021.2

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  62. Two distinct mechanisms of neuropathy in immunoglobulin light chain (AL) amyloidosis.

    Koike H, Mouri N, Fukami Y, Iijima M, Matsuo K, Yagi N, Saito A, Nakamura H, Takahashi K, Nakae Y, Okada Y, Tanaka F, Sobue G, Katsuno M

    Journal of the neurological sciences   Vol. 421   page: 117305   2021.2

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    DOI: 10.1016/j.jns.2020.117305

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  63. Association Between IL-5 Levels and the Clinicopathologic Features of Eosinophilic Granulomatosis With Polyangiitis

    Nishi Ryoji, Koike Haruki, Ohyama Ken, Fukami Yuki, Iijima Masahiro, Sobue Gen, Katsuno Masahisa

    NEUROLOGY   Vol. 96 ( 5 ) page: 226 - 229   2021.2

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  64. Severe myasthenia gravis with anti-LRP4 antibodies and Hodgkin lymphoma

    Hayashi Naoki, Sone Jun, Fukami Yuki, Yoshida Yusuke, Kuno Shunsuke, Shimada Kazuyuki, Atsuta Naoki, Nakamura Tomohiko, Higuchi Osamu, Katsuno Masahisa

    MUSCLE & NERVE   Vol. 63 ( 1 ) page: E2 - E4   2021.1

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  65. A Neuromuscular-Based Analysis of the Open Payments Program.

    Harada Y, Sheng S, Thombre VA, Ounpraseuth S, Tommee CG, Annapureddy AR, Kamran M, Jasti MS, Katsuno M, Yadala S, Veerapaneni K, Kapoor N, Kovvuru S, Onteddu SR, Nalleballe K

    Muscle & nerve   Vol. 63 ( 1 ) page: 96 - 99   2021.1

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    DOI: 10.1002/mus.27016

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  66. Genetic and functional analysis of KIF5A variants in Japanese patients with sporadic amyotrophic lateral sclerosis.

    Nakamura R, Tohnai G, Atsuta N, Nakatochi M, Hayashi N, Watanabe H, Yokoi D, Watanabe H, Katsuno M, Izumi Y, Taniguchi A, Kanai K, Morita M, Kano O, Kuwabara S, Oda M, Abe K, Aoki M, Aiba I, Okamoto K, Mizoguchi K, Hattori N, Nakashima K, Kaji R, Sobue G, Japanese Consortium for Amyotrophic Lateral Sclerosis Research (JaCALS).

    Neurobiology of aging   Vol. 97   page: 147.e11 - 147.e17   2021.1

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    DOI: 10.1016/j.neurobiolaging.2020.07.010

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  67. Duchenne Muscular Dystrophy Successfully Treated with Aripiprazole in a Patient with Autism Spectrum Disorder Symptoms Including Irritability

    Noda Seiya, Murakami Ayuka, Kimura Seigo, Minamiyama Makoto, Katsuno Masahisa, Kuru Satoshi

    INTERNAL MEDICINE   Vol. 60 ( 24 ) page: 3983 - 3986   2021

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  68. Effects of Head Motion on the Evaluation of Age-related Brain Network Changes Using Resting State Functional MRI

    Kato Sanae, Bagarinao Epifanio, Isoda Haruo, Koyama Shuji, Watanabe Hirohisa, Maesawa Satoshi, Mori Daisuke, Hara Kazuhiro, Katsuno Masahisa, Hoshiyama Minoru, Naganawa Shinji, Ozaki Norio, Sobue Gen

    MAGNETIC RESONANCE IN MEDICAL SCIENCES   Vol. 20 ( 4 ) page: 338 - 346   2021

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  69. Characteristics of Neural Network Changes in Normal Aging and Early Dementia.

    Watanabe H, Bagarinao E, Maesawa S, Hara K, Kawabata K, Ogura A, Ohdake R, Shima S, Mizutani Y, Ueda A, Ito M, Katsuno M, Sobue G

    Frontiers in aging neuroscience   Vol. 13   page: 747359   2021

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    DOI: 10.3389/fnagi.2021.747359

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  70. Longitudinal analysis of premotor anthropometric and serological markers of Parkinson's disease

    Yokoi Katsunori, Hattori Makoto, Satake Yuki, Tanaka Yasuhiro, Sato Maki, Hashizume Atsushi, Hori Akihiro, Kawashima Motoshi, Hirakawa Akihiro, Watanabe Hirohisa, Katsuno Masahisa

    SCIENTIFIC REPORTS   Vol. 10 ( 1 ) page: 20524   2020.12

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  71. Transthyretin Amyloidosis: Update on the Clinical Spectrum, Pathogenesis, and Disease-Modifying Therapies

    Koike Haruki, Katsuno Masahisa

    NEUROLOGY AND THERAPY   Vol. 9 ( 2 ) page: 317 - 333   2020.12

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  72. Association of serum neurofilament light chain levels with clinicopathology and NF155 autoantibodies in patients with CIDP

    Fukami Yuki, Iijima Masahiro, Koike Haruki, Nishi Ryoji, Hashizume Atsushi, Katsuno Masahisa

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 25 ( 4 ) page: 464 - 465   2020.12

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  73. Pathophysiology of Chronic Inflammatory Demyelinating Polyneuropathy: Insights into Classification and Therapeutic Strategy

    Koike Haruki, Katsuno Masahisa

    NEUROLOGY AND THERAPY   Vol. 9 ( 2 ) page: 213 - 227   2020.12

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    DOI: 10.1007/s40120-020-00190-8

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  74. Aberrant Expression of Nodal and Paranodal Molecules in Neuropathy Associated With IgM Monoclonal Gammopathy With Anti-Myelin-Associated Glycoprotein Antibodies.

    Kawagashira Y, Koike H, Takahashi M, Ohyama K, Iijima M, Katsuno M, Niwa JI, Doyu M, Sobue G

    Journal of neuropathology and experimental neurology   Vol. 79 ( 12 ) page: 1303 - 1312   2020.12

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    DOI: 10.1093/jnen/nlaa085

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  75. Identifying the brain's connector hubs at the voxel level using functional connectivity overlap ratio

    Bagarinao Epifanio, Watanabe Hirohisa, Maesawa Satoshi, Mori Daisuke, Hara Kazuhiro, Kawabata Kazuya, Ohdake Reiko, Masuda Michihito, Ogura Aya, Kato Toshiyasu, Koyama Shuji, Katsuno Masahisa, Wakabayashi Toshihiko, Kuzuya Masafumi, Hoshiyama Minoru, Isoda Haruo, Naganawa Shinji, Ozaki Norio, Sobue Gen

    NEUROIMAGE   Vol. 222   page: 117241   2020.11

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  76. The neural network basis of altered decision-making in patients with amyotrophic lateral sclerosis

    Imai Kazunori, Masuda Michihito, Watanabe Hirohisa, Ogura Aya, Ohdake Reiko, Tanaka Yasuhiro, Kato Toshiyasu, Kawabata Kazuya, Riku Yuichi, Hara Kazuhiro, Nakamura Ryoichi, Atsuta Naoki, Bagarinao Epifanio, Katahira Kentaro, Ohira Hideki, Katsuno Masahisa, Sobue Gen

    ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY   Vol. 7 ( 11 ) page: 2115 - 2126   2020.11

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  77. Cerebello-basal ganglia connectivity fingerprints related to motor/cognitive performance in Parkinson's disease.

    Kawabata K, Watanabe H, Bagarinao E, Ohdake R, Hara K, Ogura A, Masuda M, Kato T, Tsuboi T, Maesawa S, Katsuno M, Sobue G

    Parkinsonism & related disorders   Vol. 80   page: 21 - 27   2020.11

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    DOI: 10.1016/j.parkreldis.2020.09.005

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  78. Aging Impacts the Overall Connectivity Strength of Regions Critical for Information Transfer Among Brain Networks

    Bagarinao Epifanio, Watanabe Hirohisa, Maesawa Satoshi, Mori Daisuke, Hara Kazuhiro, Kawabata Kazuya, Yoneyama Noritaka, Ohdake Reiko, Imai Kazunori, Masuda Michihito, Yokoi Takamasa, Ogura Aya, Taoka Toshiaki, Koyama Shuji, Tanabe Hiroki C., Katsuno Masahisa, Wakabayashi Toshihiko, Kuzuya Masafumi, Hoshiyama Minoru, Isoda Haruo, Naganawa Shinji, Ozaki Norio, Sobue Gen

    FRONTIERS IN AGING NEUROSCIENCE   Vol. 12   page: 592469   2020.10

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  79. Aggresome formation and liquid-liquid phase separation independently induce cytoplasmic aggregation of TAR DNA-binding protein 43

    Watanabe Seiji, Inami Hidekazu, Oiwa Kotaro, Murata Yuri, Sakai Shohei, Komine Okiru, Sobue Akira, Iguchi Yohei, Katsuno Masahisa, Yamanaka Koji

    CELL DEATH & DISEASE   Vol. 11 ( 10 ) page: 909   2020.10

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  80. Individual changes in visual performance in non-demented Parkinson's disease patients: a 1-year follow-up study

    Ohdake Reiko, Kawabata Kazuya, Watanabe Hirohisa, Hara Kazuhiro, Ogura Aya, Kato Toshiyasu, Masuda Michihito, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEURAL TRANSMISSION   Vol. 127 ( 10 ) page: 1387 - 1397   2020.10

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  81. Combination molecular therapies for type 1 spinal muscular atrophy.

    Harada Y, Rao VK, Arya K, Kuntz NL, DiDonato CJ, Napchan-Pomerantz G, Agarwal A, Stefans V, Katsuno M, Veerapandiyan A

    Muscle & nerve   Vol. 62 ( 4 ) page: 550 - 554   2020.10

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    DOI: 10.1002/mus.27034

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  82. Disease mechanism, biomarker and therapeutics for spinal and bulbar muscular atrophy (SBMA).

    Hashizume A, Fischbeck KH, Pennuto M, Fratta P, Katsuno M

    Journal of neurology, neurosurgery, and psychiatry   Vol. 91 ( 10 ) page: 1085 - 1091   2020.10

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    DOI: 10.1136/jnnp-2020-322949

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  83. Difference in cardiovascular response during orthostatic stress in Parkinson's disease and multiple system atrophy

    Nakamura Tomohiko, Suzuki Masashi, Ueda Masamichi, Harada Yumiko, Hirayama Masaaki, Katsuno Masahisa

    JOURNAL OF NEURAL TRANSMISSION   Vol. 127 ( 10 ) page: 1377 - 1386   2020.10

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    DOI: 10.1007/s00702-020-02241-8

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  84. A multi-ethnic meta-analysis identifies novel genes, including ACSL5, associated with amyotrophic lateral sclerosis.

    Nakamura R, Misawa K, Tohnai G, Nakatochi M, Furuhashi S, Atsuta N, Hayashi N, Yokoi D, Watanabe H, Watanabe H, Katsuno M, Izumi Y, Kanai K, Hattori N, Morita M, Taniguchi A, Kano O, Oda M, Shibuya K, Kuwabara S, Suzuki N, Aoki M, Ohta Y, Yamashita T, Abe K, Hashimoto R, Aiba I, Okamoto K, Mizoguchi K, Hasegawa K, Okada Y, Ishihara T, Onodera O, Nakashima K, Kaji R, Kamatani Y, Ikegawa S, Momozawa Y, Kubo M, Ishida N, Minegishi N, Nagasaki M, Sobue G

    Communications biology   Vol. 3 ( 1 ) page: 526   2020.9

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    DOI: 10.1038/s42003-020-01251-2

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  85. Patisiran, an RNAi therapeutic for patients with hereditary transthyretin-mediated amyloidosis: Sub-analysis in Japanese patients from the APOLLO study

    Yamashita Taro, Ueda Mitsuharu, Koike Haruki, Sekijima Yoshiki, Yoshinaga Tsuneaki, Kodaira Minori, Katsuno Masahisa, Sobue Gen, Zhang Xiaoping, White Matthew T., Sweetser Marianne T., Wang Jing Jing, Ando Yukio

    NEUROLOGY AND CLINICAL NEUROSCIENCE   Vol. 8 ( 5 ) page: 251 - 260   2020.9

  86. Meta-Analysisof Gut Dysbiosis in Parkinson's Disease

    Nishiwaki Hiroshi, Ito Mikako, Ishida Tomohiro, Hamaguchi Tomonari, Maeda Tetsuya, Kashihara Kenichi, Tsuboi Yoshio, Ueyama Jun, Shimamura Teppei, Mori Hiroshi, Kurokawa Ken, Katsuno Masahisa, Hirayama Masaaki, Ohno Kinji

    MOVEMENT DISORDERS   Vol. 35 ( 9 ) page: 1626 - 1635   2020.9

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    DOI: 10.1002/mds.28119

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  87. Later-Onset Multiple System Atrophy: A Multicenter Asian Study.

    Lee YH, Ando T, Lee JJ, Baek MS, Lyoo CH, Kim SJ, Kim M, Cho JW, Sohn YH, Katsuno M, Watanabe H, Yoshida M, Lee PH

    Movement disorders : official journal of the Movement Disorder Society   Vol. 35 ( 9 ) page: 1692 - 1693   2020.9

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    DOI: 10.1002/mds.28177

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  88. A nerve conduction study predicts the prognosis of sporadic amyotrophic lateral sclerosis

    Imai Eriko, Nakamura Tomohiko, Atsuta Naoki, Nakatochi Masahiro, Suzuki Masashi, Harada Yumiko, Nakamura Ryoichi, Hayashi Naoki, Sobue Gen, Katsuno Masahisa

    JOURNAL OF NEUROLOGY   Vol. 267 ( 9 ) page: 2524 - 2532   2020.9

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    DOI: 10.1007/s00415-020-09858-5

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  89. Therapeutic monoclonal antibody targeting of neuronal pentraxin receptor to control metastasis in gastric cancer

    Kanda Mitsuro, Shimizu Dai, Sawaki Koichi, Nakamura Shunsuke, Umeda Shinichi, Miwa Takashi, Tanaka Haruyoshi, Tanaka Chie, Hayashi Masamichi, Iguchi Yohei, Yamada Suguru, Katsuno Masahisa, Kodera Yasuhiro

    MOLECULAR CANCER   Vol. 19 ( 1 ) page: 131   2020.8

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  90. Changes in white matter fiber density and morphology across the adult lifespan: A cross-sectional fixel-based analysis

    Choy Shao Wei, Bagarinao Epifanio, Watanabe Hirohisa, Ho Eric Tatt Wei, Maesawa Satoshi, Mori Daisuke, Hara Kazuhiro, Kawabata Kazuya, Yoneyama Noritaka, Ohdake Reiko, Imai Kazunori, Masuda Michihito, Yokoi Takamasa, Ogura Aya, Taoka Toshiaki, Koyama Shuji, Tanabe Hiroki C., Katsuno Masahisa, Wakabayashi Toshihiko, Kuzuya Masafumi, Hoshiyama Minoru, Isoda Haruo, Naganawa Shinji, Ozaki Norio, Sobue Gen

    HUMAN BRAIN MAPPING   Vol. 41 ( 12 ) page: 3198 - 3211   2020.8

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    DOI: 10.1002/hbm.25008

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  91. Aberrant interaction between FUS and SFPQ in neurons in a wide range of FTLD spectrum diseases.

    Ishigaki S, Riku Y, Fujioka Y, Endo K, Iwade N, Kawai K, Ishibashi M, Yokoi S, Katsuno M, Watanabe H, Mori K, Akagi A, Yokota O, Terada S, Kawakami I, Suzuki N, Warita H, Aoki M, Yoshida M, Sobue G

    Brain : a journal of neurology   Vol. 143 ( 8 ) page: 2398 - 2405   2020.8

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    DOI: 10.1093/brain/awaa196

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  92. Aberrant interaction between FUS and SFPQ in neurons in a wide range of FTLD spectrum diseases

    Ishigaki Shinsuke, Riku Yuichi, Fujioka Yusuke, Endo Kuniyuki, Iwade Nobuyuki, Kawai Kaori, Ishibashi Minaka, Yokoi Satoshi, Katsuno Masahisa, Watanabe Hirohisa, Mori Keiko, Akagi Akio, Yokota Osamu, Terada Seishi, Kawakami Ito, Suzuki Naoki, Warita Hitoshi, Aoki Masashi, Yoshida Mari, Sobue Gen

    BRAIN   Vol. 143   page: 2398 - 2405   2020.8

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  93. NUS1 mutation in a family with epilepsy, cerebellar ataxia, and tremor

    Araki Kunihiko, Nakamura Ryoichi, Ito Daisuke, Kato Kohji, Iguchi Yohei, Sahashi Kentaro, Toyama Miho, Hamada Kensuke, Okamoto Nobuhiko, Wada Yoshinao, Nakamura Tomohiko, Ogi Tomoo, Katsuno Masahisa

    EPILEPSY RESEARCH   Vol. 164   page: 106371   2020.8

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    DOI: 10.1016/j.eplepsyres.2020.106371

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  94. Muscle BDNF improves synaptic and contractile muscle strength in Kennedy's disease mice in a muscle-type specific manner

    Halievski Katherine, Xu Youfen, Haddad Yazeed W., Tang Yu Ping, Yamada Shinichiro, Katsuno Masahisa, Adachi Hiroaki, Sobue Gen, Breedlove S. Marc, Jordan Cynthia L.

    JOURNAL OF PHYSIOLOGY-LONDON   Vol. 598 ( 13 ) page: 2719 - 2739   2020.7

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    DOI: 10.1113/JP279208

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  95. [Relationship between Drug Metabolism and Intestinal Microbiota in Parkinson's Disease].

    Hirayama M, Ohno K, Katsuno M

    Brain and nerve = Shinkei kenkyu no shinpo   Vol. 72 ( 7 ) page: 805 - 809   2020.7

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    DOI: 10.11477/mf.1416201599

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  96. Arginine is a disease modifier for polyQ disease models that stabilizes polyQ protein conformation

    Minakawa Eiko N., Popiel Helena Akiko, Tada Masayoshi, Takahashi Toshiaki, Yamane Hiroshi, Saitoh Yuji, Takahashi Yasuo, Ozawa Daisaku, Takeda Akiko, Takeuchi Toshihide, Okamoto Yuma, Yamamoto Kazuhiro, Suzuki Mari, Fujita Hiromi, Ito Chiyomi, Yagihara Hiroko, Saito Yuko, Watase Kei, Adachi Hiroaki, Katsuno Masahisa, Mochizuki Hideki, Shiraki Kentaro, Sobue Gen, Toda Tatsushi, Wada Keiji, Onodera Osamu, Nagai Yoshitaka

    BRAIN   Vol. 143 ( 6 ) page: 1811 - 1825   2020.6

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    DOI: 10.1093/brain/awaa115

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  97. Relationship between cardiac parasympathetic dysfunction and the anteroposterior diameter of the medulla oblongata in multiple system atrophy

    Suzuki Masashi, Nakamura Tomohiko, Hirayama Masaaki, Ueda Miki, Imai Eriko, Harada Yumiko, Katsuno Masahisa

    CLINICAL AUTONOMIC RESEARCH   Vol. 30 ( 3 ) page: 231 - 238   2020.6

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    DOI: 10.1007/s10286-020-00675-4

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  98. Steroid-responsive recurrent tumefactive demyelination with multiple petechial hemorrhages along non-displaced medullary veins

    Tsuboi Takashi, Harada Yumiko, Suzuki Masashi, Ando Takashi, Atsuta Naoki, Ohka Fumiharu, Takeuchi Kazuhito, Taoka Toshiaki, Ohba Shigeo, Nakaguro Masato, Abe Masato, Nakashima Ichiro, Yoshida Mari, Katsuno Masahisa

    CLINICAL NEUROLOGY AND NEUROSURGERY   Vol. 193   page: 105764   2020.6

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    DOI: 10.1016/j.clineuro.2020.105764

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  99. Ultrastructural mechanisms of macrophage-induced demyelination in Guillain-Barre syndrome

    Koike Haruki, Fukami Yuki, Nishi Ryoji, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY   Vol. 91 ( 6 ) page: 650 - 659   2020.6

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    DOI: 10.1136/jnnp-2019-322479

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  100. Oral Venglustat in Parkinson's Disease Patients With a GBA Mutation: Part 1 Baseline Characteristics and Results and Part 2 Study Design of the MOVES-PD Trial

    Peterschmitt M. J., Hattori N., Saiki H., Hatano T., Gasser T., Isaacson S., Kulisevsky J., Mir P., Simuni T., Takahashi Y., Wills A. -M., Correia Guedes L., Svenningsson P., Takahashi R., Waters C., Katsuno M., Ji A. J., Gaemers S. J. M., Minini P., Sardi S. P., Saubadu S., Fischer T.

    EUROPEAN JOURNAL OF NEUROLOGY   Vol. 27   page: 321 - 321   2020.5

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  101. Subjects at risk of Parkinson's disease in health checkup examinees: cross-sectional analysis of baseline data of the NaT-PROBE study

    Hattori Makoto, Tsuboi Takashi, Yokoi Katsunori, Tanaka Yasuhiro, Sato Maki, Suzuki Keisuke, Arahata Yutaka, Hori Akihiro, Kawashima Motoshi, Hirakawa Akihiro, Washimi Yukihiko, Watanabe Hirohisa, Katsuno Masahisa

    JOURNAL OF NEUROLOGY   Vol. 267 ( 5 ) page: 1516 - 1526   2020.5

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    DOI: 10.1007/s00415-020-09714-6

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  102. Association of orthostatic blood pressure with the symptoms of orthostatic hypotension and cognitive impairment in patients with multiple system atrophy

    Ueda Miki, Nakamura Tomohiko, Suzuki Masashi, Imai Eriko, Harada Yumiko, Hara Kazuhiro, Hirayama Masaaki, Katsuno Masahisa

    JOURNAL OF CLINICAL NEUROSCIENCE   Vol. 75   page: 40 - 44   2020.5

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    DOI: 10.1016/j.jocn.2020.03.040

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  103. Differential clinicopathologic features of EGPA-associated neuropathy with and without ANCA

    Nishi Ryoji, Koike Haruki, Ohyama Ken, Fukami Yuki, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    NEUROLOGY   Vol. 94 ( 16 ) page: E1726 - E1737   2020.4

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    DOI: 10.1212/WNL.0000000000009309

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  104. Src Inhibition Attenuates Polyglutamine-mediated Neuromuscular Degeneration in Spinal and Bulbar Muscular Atrophy

    Iida Madoka, Sahashi Kentaro, Kondo Naohide, Nakatsuji Hideaki, Tohnai Genki, Katsuno Masahisa

    NEUROLOGY   Vol. 94 ( 15 )   2020.4

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  105. Japan Consortium of Ataxias (J-CAT): A National Registry to Elucidate Landscape for Degenerative Ataxias in Japan

    Takahashi Yuji, Date Hidetoshi, Hama Yuka, Oda Shinji, Ishikawa Kinya, Ugawa Yoshikazu, Onodera Osamu, Kira Jun-ichi, Kuwabara Satoshi, Sasaki Hidenao, Katsuno Masahisa, Sobue Gen, Takashima Hiroshi, Takiyama Yoshihisa, Takeda Atsushi, Tsuji Shoji, Hanajima Ritsuko, Nakashima Kenji, Miyai Ichiro, Yoshida Kunihiro, Mizusawa Hidehiro

    NEUROLOGY   Vol. 94 ( 15 )   2020.4

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  106. Perampanel for Sporadic Amyotrophic Lateral Sclerosis

    Aizawa Hitoshi, Kato Haruhisa, Oba Koji, Kawahara Takuya, Okubo Yoshihiko, Saito Tomoko, Urushitani Makoto, Tamaoka Akira, Nakamagoe Kiyotaka, Ishii Kazuhiro, Kanda Takashi, Katsuno Masahisa, Atsuta Naoki, Maeda Yasushi, Nagai Makiko, Nishiyama Kazutoshi, Ishiura Hiroyuki, Toda Tatsushi, Kawata Akihiro, Abe Koji, Yabe Ichiro, Takahashi-Iwata Ikuko, Sasaki Hidenao, Warita Hitoshi, Aoki Masashi, Sobue Gen, Mizusawa Hidehiro, Matsuyama Yutaka, Kwak Shin

    NEUROLOGY   Vol. 94 ( 15 )   2020.4

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  107. Safety, Pharmacokinetics, and Pharmacodynamics of Oral Venglustat in the Japanese and the Rest of the World Parkinson's Disease Population With a GBA Mutation: Results from Part 1 of the MOVES-PD Study

    Peterschmitt M. Judith, Saiki Hidemoto, Hatano Taku, Gasser Thomas, Isaacson Stuart, Kulisevsky Jaime, Simuni Tanya, Takahashi Yuji, Wills Anne-Marie, Guedes Leonor Correia, Svenningsson Per, Takahashi Ryosuke, Waters Cheryl, Katsuno Masahisa, Ji Allena Ji, Gaemers Sebastiaan J. M., Minini Pascal, Nembo Blandine, Sardi Pablo, Saubadu Stephane, Sharma Jyoti, Fischer Tanya

    NEUROLOGY   Vol. 94 ( 15 )   2020.4

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  108. Efficacy and Safety of Rituximab in Refractory CIDP With or Without IgG4 Autoantibodies (RECIPE): Protocol for a Double-Blind, Randomized, Placebo-Controlled Clinical Trial

    Shimizu Shinobu, Iijima Masahiro, Fukami Yuki, Tamura Natsuko, Nakatochi Masahiro, Ando Masahiko, Nishi Ryoji, Koike Haruki, Kaida Kenichi, Koga Michiaki, Kanda Takashi, Ogata Hidenori, Kira Jun-Ichi, Mori Masahiro, Kuwabara Satoshi, Katsuno Masahisa

    JMIR RESEARCH PROTOCOLS   Vol. 9 ( 4 ) page: e17117   2020.4

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    DOI: 10.2196/17117

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  109. Characteristic Features of FUS Inclusions in Spinal Motor Neurons of Sporadic Amyotrophic Lateral Sclerosis

    Ikenaka Kensuke, Ishigaki Shinsuke, Iguchi Yohei, Kawai Kaori, Fujioka Yusuke, Yokoi Satoshi, Abdelhamid Rehab F., Nagano Seiichi, Mochizuki Hideki, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY   Vol. 79 ( 4 ) page: 370 - 377   2020.4

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    DOI: 10.1093/jnen/nlaa003

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  110. Impact of orthostatic hypotension on wheelchair use in patients with Parkinson's disease

    Nakamura Tomohiko, Suzuki Masashi, Ueda Masamichi, Harada Yumiko, Hirayama Masaaki, Katsuno Masahisa

    JOURNAL OF NEURAL TRANSMISSION   Vol. 127 ( 3 ) page: 379 - 383   2020.3

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    DOI: 10.1007/s00702-019-02127-4

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  111. Prognosis of amyotrophic lateral sclerosis patients undergoing tracheostomy invasive ventilation therapy in Japan

    Hayashi Naoki, Atsuta Naoki, Yokoi Daichi, Nakamura Ryoichi, Nakatochi Masahiro, Katsuno Masahisa, Izumi Yuishin, Kanai Kazuaki, Hattori Nobutaka, Taniguchi Akira, Morita Mitsuya, Kano Osamu, Shibuya Kazumoto, Kuwabara Satoshi, Suzuki Naoki, Aoki Masashi, Aiba Ikuko, Mizoguchi Kouichi, Oda Masaya, Kaji Ryuji, Sobue Gen

    JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY   Vol. 91 ( 3 ) page: 285 - 290   2020.3

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    DOI: 10.1136/jnnp-2019-322213

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  112. Unveiling synapse pathology in spinal bulbar muscular atrophy by genome-wide transcriptome analysis of purified motor neurons derived from disease specific iPSCs

    Onodera Kazunari, Shimojo Daisuke, Ishihara Yasuharu, Yano Masato, Miya Fuyuki, Banno Haruhiko, Kuzumaki Naoko, Ito Takuji, Okada Rina, de Araujo Herculano Bruno, Ohyama Manabu, Yoshida Mari, Tsunoda Tatsuhiko, Katsuno Masahisa, Doyu Manabu, Sobue Gen, Okano Hideyuki, Okada Yohei

    MOLECULAR BRAIN   Vol. 13 ( 1 ) page: 18   2020.2

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    DOI: 10.1186/s13041-020-0561-1

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  113. Safety, pharmacokinetics, and pharmacodynamics of oral venglustat in Parkinson disease patients with a GBA mutation from Japan and the rest of the world: Results from part 'I of the MOVES-PD study

    Peterschmitt M. Judith, Saiki Hidemoto, Hatano Taku, Gasser Thomas, Isaacson Stuart, Kulisevsky Jaime, Simuni Tanya, Takahashi Yuji, Wills Anne-Marie, Guedes Leonor Correia, Svenningsson Per, Takahashi Ryosuke, Waters Cheryl, Katsuno Masahisa, Ji Allena Ji, Gaemers Sebastiaan J. M., Minini Pascal, Nembo Blandine, Sardi Pablo, Saubadu Stephane, Sharma Jyoti, Fischer Tanya

    MOLECULAR GENETICS AND METABOLISM   Vol. 129 ( 2 ) page: S128 - S128   2020.2

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    DOI: 10.1016/j.ymgme.2019.11.334

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  114. Pharmacological monitoring of antiepileptic drugs in epilepsy patients on haemodialysis

    Araki Kunihiko, Nakamura Tomohiko, Takeuchi Yuko, Morozumi Saori, Horie Katsunori, Kobayashi Yasushi, Kawakami Osamu, Sobue Fumio, Ueda Takuya, Hamada Kensuke, Ando Tetsuo, Inoue Yushi, Yasui Keizo, Morozumi Kunio, Maruyama Shoichi, Katsuno Masahisa

    EPILEPTIC DISORDERS   Vol. 22 ( 1 ) page: 90 - 102   2020.2

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    DOI: 10.1684/epd.2020.1139

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  115. YAP-dependent necrosis occurs in early stages of Alzheimer's disease and regulates mouse model pathology.

    Tanaka H, Homma H, Fujita K, Kondo K, Yamada S, Jin X, Waragai M, Ohtomo G, Iwata A, Tagawa K, Atsuta N, Katsuno M, Tomita N, Furukawa K, Saito Y, Saito T, Ichise A, Shibata S, Arai H, Saido T, Sudol M, Muramatsu SI, Okano H, Mufson EJ, Sobue G, Murayama S, Okazawa H

    Nature communications   Vol. 11 ( 1 ) page: 507   2020.1

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    DOI: 10.1038/s41467-020-14353-6

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  116. Complement deposition and macrophage-induced demyelination in CIDP with anti-LM1 antibodies

    Koike Haruki, Ikeda Shohei, Fukami Yuki, Nishi Ryoji, Kawagashira Yuichi, Iijima Masahiro, Nakamura Tomohiko, Kuwahara Motoi, Kusunoki Susumu, Katsuno Masahisa, Sobue Gen

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 408   page: 116509   2020.1

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    DOI: 10.1016/j.jns.2019.116509

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  117. Expanding the spectrum of transthyretin amyloidosis

    Koike Haruki, Katsuno Masahisa

    MUSCLE & NERVE   Vol. 61 ( 1 ) page: 3 - 4   2020.1

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    DOI: 10.1002/mus.26741

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  118. Longitudinal Speech Change After Subthalamic Nucleus Deep Brain Stimulation in Parkinson's Disease Patients: A 2-Year Prospective Study

    Tanaka Yasuhiro, Tsuboi Takashi, Watanabe Hirohisa, Nakatsubo Daisuke, Maesawa Satoshi, Kato Sachiko, Kajita Yasukazu, Sato Maki, Oodake Reiko, Hattori Makoto, Yamamoto Masahiko, Wakabayashi Toshihiko, Katsuno Masahisa, Sobue Gen

    JOURNAL OF PARKINSONS DISEASE   Vol. 10 ( 1 ) page: 131 - 140   2020

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    DOI: 10.3233/JPD-191798

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  119. Longitudinal Change of DAT SPECT in Parkinson's Disease and Multiple System Atrophy

    Sakakibara Satoko, Hashimoto Rina, Katayama Taiji, Kenjyo Masakuni, Yokokawa Yuki, Saito Yufuko, Hirakawa Akihiro, Ito Mizuki, Nakamura Tomohiko, Hara Kazuhiro, Hashizume Atsushi, Aiba Ikuko, Inukai Akira, Katsuno Masahisa

    JOURNAL OF PARKINSONS DISEASE   Vol. 10 ( 1 ) page: 123 - 130   2020

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    DOI: 10.3233/JPD-191710

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  120. Short-Chain Fatty Acid-Producing Gut Microbiota Is Decreased in Parkinson's Disease but Not in Rapid-Eye-Movement Sleep Behavior Disorder

    Nishiwaki Hiroshi, Hamaguchi Tomonari, Ito Mikako, Ishida Tomohiro, Maeda Tetsuya, Kashihara Kenichi, Tsuboi Yoshio, Ueyama Jun, Shimamura Teppei, Mori Hiroshi, Kurokawa Ken, Katsuno Masahisa, Hirayama Masaaki, Ohnoa Kinji

    MSYSTEMS   Vol. 5 ( 6 )   2020

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  121. Visuoperceptual disturbances in Parkinson's disease.

    Kawabata K, Ohdake R, Watanabe H, Bagarinao E, Hara K, Ogura A, Masuda M, Kato T, Yokoi T, Katsuno M, Sobue G

    Clinical parkinsonism & related disorders   Vol. 3   page: 100036   2020

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    DOI: 10.1016/j.prdoa.2020.100036

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  122. [Late phase II/III study of BYM338 in patients with sporadic inclusion body myositis (RESILIENT): Japanese cohort data].

    Mori-Yoshimura M, Yamashita S, Suzuki N, Katsuno M, Murata K, Nodera H, Teshima R, Inamura T, Nishino I, Aoki M

    Rinsho shinkeigaku = Clinical neurology   Vol. 59 ( 12 ) page: 806-813   2019.12

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    DOI: 10.5692/clinicalneurol.cn-001325

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  123. Nasometric Scores in spinal and bulbar muscular atrophy - Effects of palatal lift prosthesis on dysarthria and dysphagia

    Tanaka Seiya, Hashizume Atsushi, Hijikata Yasuhiro, Yamada Shinichiro, Ito Daisuke, Nakayama Atsushi, Kurita Kenichi, Yogo Hiroaki, Banno Haruhiko, Suzuki Keisuke, Yamamoto Masahiko, Sobue Gen, Katsuno Masahisa

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 407   page: 116503   2019.12

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    DOI: 10.1016/j.jns.2019.116503

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  124. Elevated serum creatine kinase in the early stage of sporadic amyotrophic lateral sclerosis. Reviewed

    Ito D, Hashizume A, Hijikata Y, Yamada S, Iguchi Y, Iida M, Kishimoto Y, Moriyoshi H, Hirakawa A, Katsuno M

    Journal of neurology   Vol. 266 ( 12 ) page: 2952 - 2961   2019.12

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    DOI: 10.1007/s00415-019-09507-6

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  125. Increased prevalence of granulovacuolar degeneration in C9orf72 mutation. Reviewed

    Riku Y, Duyckaerts C, Boluda S, Plu I, Le Ber I, Millecamps S, Salachas F, Brainbank NeuroCEB Neuropathology Network., Yoshida M, Ando T, Katsuno M, Sobue G, Seilhean D

    Acta neuropathologica   Vol. 138 ( 5 ) page: 783 - 793   2019.11

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    DOI: 10.1007/s00401-019-02028-6

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  126. [The Role of Patient Registry in the Care and Therapeutic Development for Patients with Amyotrophic Lateral Sclerosis: JaCALS].

    Atsuta N, Nakamura R, Hayashi N, Tohnai G, Katsuno M, Sobue G

    Brain and nerve = Shinkei kenkyu no shinpo   Vol. 71 ( 11 ) page: 1215-1225   2019.11

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    DOI: 10.11477/mf.1416201431

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  127. Demyelinating Neuropathy Due to Intravascular Large B-cell Lymphoma: A Case Report. Reviewed

    Fukami Y, Koike H, Iijima M, Hagita J, Niwa H, Nishi R, Kawagashira Y, Katsuno M

    Internal medicine (Tokyo, Japan)     2019.10

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    DOI: 10.2169/internalmedicine.3228-19

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  128. [Multiple mononeuropathy associated with systemic sclerosis with vasculitis confirmed by nerve biopsy: a case report].

    Kano Y, Kato H, Koike H, Katsuno M, Oguri T, Yuasa H

    Rinsho shinkeigaku = Clinical neurology   Vol. 59 ( 9 ) page: 604 - 606   2019.9

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    DOI: 10.5692/clinicalneurol.cn-001320

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  129. Src inhibition attenuates polyglutamine-mediated neuromuscular degeneration in spinal and bulbar muscular atrophy Reviewed

    Iida Madoka, Sahashi Kentaro, Kondo Naohide, Nakatsuji Hideaki, Tohnai Genki, Tsutsumi Yutaka, Noda Seiya, Murakami Ayuka, Onodera Kazunari, Okada Yohei, Nakatochi Masahiro, Okabe Yuka Tsukagoshi, Shimizu Shinobu, Mizuno Masaaki, Adachi Hiroaki, Okano Hideyuki, Sobue Gen, Katsuno Masahisa

    NATURE COMMUNICATIONS   Vol. 10 ( 1 ) page: 4262   2019.9

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    DOI: 10.1038/s41467-019-12282-7

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  130. Safety and efficacy of intravenous bimagrumab in inclusion body myositis (RESILIENT): a randomised, double-blind, placebo-controlled phase 2b trial Reviewed

    Hanna Michael G., Badrising Umesh A., Benveniste Olivier, Lloyd Thomas E., Needham Merrilee, Chinoy Hector, Aoki Masashi, Machado Pedro M., Liang Christina, Reardon Katrina A., de Visser Marianne, Ascherman Dana P., Barohn Richard J., Dimachkie Mazen M., Miller James A. L., Kissel John T., Oskarsson Bjoern, Joyce Nanette C., Van den Bergh Peter, Baets Jonathan, De Bleecker Jan L., Karam Chafic, David William S., Mirabella Massimiliano, Nations Sharon P., Jung Hans H., Pegoraro Elena, Maggi Lorenzo, Rodolico Carmelo, Filosto Massimiliano, Shaibani Aziz I, Sivakumar Kumaraswamy, Goyal Namita A., Mori-Yoshimura Madoka, Yamashita Satoshi, Suzuki Naoki, Katsuno Masahisa, Murata Kenya, Nodera Hiroyuki, Romano Carla D., Williams Valerie S. L., Vissing John, Auberson Lixin Zhang, Wu Min, de Vera Ana, Papanicolaou Dimitris A., Amato Anthony A., Nishino Ichizo

    LANCET NEUROLOGY   Vol. 18 ( 9 ) page: 834 - 844   2019.9

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    DOI: 10.1016/S1474-4422(19)30200-5.

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  131. Semantic deficits in ALS related to right lingual/fusiform gyrus network involvement Reviewed

    Ogura Aya, Watanabe Hirohisa, Kawabata Kazuya, Ohdake Reiko, Tanaka Yasuhiro, Masuda Michihito, Kato Toshiyasu, Imai Kazunori, Yokoi Takamasa, Hara Kazuhiro, Bagarinao Epifanio, Riku Yuichi, Nakamura Ryoichi, Kawai Yoshinari, Nakatochi Masahiro, Atsuta Naoki, Katsuno Masahisa, Sobue Gen

    EBIOMEDICINE   Vol. 47   page: 506 - 517   2019.9

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    DOI: 10.1016/j.ebiom.2019.08.022

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  132. Safety and efficacy of intravenous bimagrumab in inclusion body myositis (RESILIENT): a randomised, double-blind, placebo-controlled phase 2b trial.

    Hanna MG, Badrising UA, Benveniste O, Lloyd TE, Needham M, Chinoy H, Aoki M, Machado PM, Liang C, Reardon KA, de Visser M, Ascherman DP, Barohn RJ, Dimachkie MM, Miller JAL, Kissel JT, Oskarsson B, Joyce NC, Van den Bergh P, Baets J, De Bleecker JL, Karam C, David WS, Mirabella M, Nations SP, Jung HH, Pegoraro E, Maggi L, Rodolico C, Filosto M, Shaibani AI, Sivakumar K, Goyal NA, Mori-Yoshimura M, Yamashita S, Suzuki N, Katsuno M, Murata K, Nodera H, Nishino I, Romano CD, Williams VSL, Vissing J, Auberson LZ, Wu M, de Vera A, Papanicolaou DA, Amato AA, RESILIENT Study Group.

    The Lancet. Neurology   Vol. 18 ( 9 ) page: 834 - 844   2019.9

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    DOI: 10.1016/S1474-4422(19)30200-5

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  133. Reorganization of brain networks and its association with general cognitive performance over the adult lifespan Reviewed

    Bagarinao Epifanio, Watanabe Hirohisa, Maesawa Satoshi, Mori Daisuke, Hara Kazuhiro, Kawabata Kazuya, Yoneyama Noritaka, Ohdake Reiko, Imai Kazunori, Masuda Michihito, Yokoi Takamasa, Ogura Aya, Taoka Toshiaki, Koyama Shuji, Tanabe Hiroki C., Katsuno Masahisa, Wakabayashi Toshihiko, Kuzuya Masafumi, Ozaki Norio, Hoshiyama Minoru, Isoda Haruo, Naganawa Shinji, Sobue Gen

    SCIENTIFIC REPORTS   Vol. 9 ( 1 ) page: 11352   2019.8

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    DOI: 10.1038/s41598-019-47922-x

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  134. Cilostazol use is associated with FIM cognitive improvement during convalescent rehabilitation in patients with ischemic stroke: a retrospective study Reviewed

    Senda Joe, Ito Keiichi, Kotake Tomomitsu, Kanamori Masahiko, Kishimoto Hideo, Kadono Izumi, Nakagawa-Senda Hiroko, Wakai Kenji, Katsuno Masahisa, Nishida Yoshihiro, Ishiguro Naoki, Sobue Gen

    NAGOYA JOURNAL OF MEDICAL SCIENCE   Vol. 81 ( 3 ) page: 359 - 373   2019.8

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    DOI: 10.18999/nagjms.81.3.359

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  135. Long-read sequencing identifies GGC repeat expansions in NOTCH2NLC associated with neuronal intranuclear inclusion disease Reviewed

    Sone Jun, Mitsuhashi Satomi, Fujita Atsushi, Mizuguchi Takeshi, Hamanaka Kohei, Mori Keiko, Koike Haruki, Hashiguchi Akihiro, Takashima Hiroshi, Sugiyama Hiroshi, Kohno Yutaka, Takiyama Yoshihisa, Maeda Kengo, Doi Hiroshi, Koyano Shigeru, Takeuchi Hideyuki, Kawamoto Michi, Kohara Nobuo, Ando Tetsuo, Ieda Toshiaki, Kita Yasushi, Kokubun Norito, Tsuboi Yoshio, Katoh Kazutaka, Kino Yoshihiro, Katsuno Masahisa, Iwasaki Yasushi, Yoshida Mari, Tanaka Fumiaki, Suzuki Ikuo K., Frith Martin C., Matsumoto Naomichi, Sobue Gen

    NATURE GENETICS   Vol. 51 ( 8 ) page: 1215 - 1221   2019.8

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    DOI: 10.1038/s41588-019-0459-y

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  136. TDP-43 regulates early-phase insulin secretion via CaV1.2-mediated exocytosis in islets Reviewed

    Araki Kunihiko, Araki Amane, Honda Daiyu, Izumoto Takako, Hashizume Atsushi, Hijikata Vasuhiro, Yamada Shinichiro, Iguchi Yohei, Hara Akitoshi, Ikumi Kazuhiro, Kawai Kaori, Ishigaki Shinsuke, Nakamichi Yoko, Tsunekawa Shin, Seino Yusuke, Yamamoto Akiko, Takayama Yasunori, Hidaka Shihomi, Tominaga Makoto, Ohara-Imaizumi Mica, Suzuki Atsushi, Ishiguro Hiroshi, Enomoto Atsushi, Yoshida Mari, Arima Hiroshi, Muramatsu Shin-ichi, Sobue Gen, Katsuno Masahisa

    JOURNAL OF CLINICAL INVESTIGATION   Vol. 129 ( 9 ) page: 3578 - 3593   2019.7

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    DOI: 10.1172/JCI124481

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  137. A behavior-based drug screening system using a Caenorhabditis elegans model of motor neuron disease Reviewed

    IKenaka Kensuke, Tsukada Yuki, Giles Andrew C., Arai Tadamasa, Nakadera Yasuhito, Nakano Shunji, Kawai Kaori, Mochizuki Hideki, Katsuno Masahisa, Sobue Gen, Mori Ikue

    SCIENTIFIC REPORTS   Vol. 9 ( 1 ) page: 10104   2019.7

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    DOI: 10.1038/s41598-019-46642-6

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  138. Clinicopathological spectrum and recent advances in the treatment of hereditary transthyretin amyloidosis Reviewed

    Koike Haruki, Fukami Yuki, Nishi Ryoji, Kawagashira Yuichi, Iijima Masahiro, Sobue Gen, Katsuno Masahisa

    NEUROLOGY AND CLINICAL NEUROSCIENCE   Vol. 7 ( 4 ) page: 166-173   2019.7

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    DOI: 10.1111/ncn3.12306

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  139. Intractable axonal neuropathy with multifocal peripheral nerve swelling in neuromyelitis optica spectrum disorders: A case report.

    Mizuno Y, Shinoda K, Watanabe M, Ogata H, Isobe N, Matsushita T, Yamasaki R, Tanaka K, Koike H, Katsuno M, Kira JI

    Multiple sclerosis and related disorders   Vol. 35   page: 16-18   2019.6

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    DOI: 10.1016/j.msard.2019.06.033

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  140. Clinicopathological characteristics of subtypes of chronic inflammatory demyelinating polyradiculoneuropathy.

    Ikeda S, Koike H, Nishi R, Kawagashira Y, Iijima M, Katsuno M, Sobue G

    Journal of neurology, neurosurgery, and psychiatry     2019.6

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    DOI: 10.1136/jnnp-2019-320741

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  141. Clinical correlates of repetitive speech disorders in Parkinson's disease.

    Tsuboi T, Watanabe H, Tanaka Y, Ohdake R, Sato M, Hattori M, Kawabata K, Hara K, Nakatsubo D, Maesawa S, Kajita Y, Katsuno M, Sobue G

    Journal of the neurological sciences   Vol. 401   page: 67 - 71   2019.6

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    DOI: 10.1016/j.jns.2019.04.012

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  142. DEFENSIVE Stroke Scale: Novel Diagnostic Tool for Predicting Posterior Circulation Infarction in the Emergency Department.

    Yamada S, Yasui K, Kawakami Y, Hasegawa Y, Katsuno M

    Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association   Vol. 28 ( 6 ) page: 1561 - 1570   2019.6

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    DOI: 10.1016/j.jstrokecerebrovasdis.2019.03.005

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  143. Inflammatory myopathy associated with PD-1 inhibitors.

    Seki M, Uruha A, Ohnuki Y, Kamada S, Noda T, Onda A, Ohira M, Isami A, Hiramatsu S, Hibino M, Nakane S, Noda S, Yutani S, Hanazono A, Yaguchi H, Takao M, Shiina T, Katsuno M, Nakahara J, Matsubara S, Nishino I, Suzuki S

    Journal of autoimmunity   Vol. 100   page: 105 - 113   2019.6

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    DOI: 10.1016/j.jaut.2019.03.005

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  144. Vasculitic neuropathy with anti-phosphatidylserine/prothrombin complex antibody

    Ando Takashi, Iijima Masahiro, Fukami Yuki, Nishi Ryoji, Ikeda Shohei, Yokoi Satoshi, Kawagashira Yuichi, Koike Haruki, Muro Yoshinao, Katsuno Masahisa

    MUSCLE & NERVE   Vol. 59 ( 6 ) page: E44 - E46   2019.6

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    DOI: 10.1002/mus.26458

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  145. Alterations in Cognition-Related Cerebello-Cerebral Networks in Multiple System Atrophy.

    Kawabata K, Hara K, Watanabe H, Bagarinao E, Ogura A, Masuda M, Yokoi T, Kato T, Ohdake R, Ito M, Katsuno M, Sobue G

    Cerebellum (London, England)     2019.5

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    DOI: 10.1007/s12311-019-01031-7

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  146. DNA methylation inhibitor attenuates polyglutamine-induced neurodegeneration by regulating Hes5

    Kondo Naohide, Tohnai Genki, Sahashi Kentaro, Iida Madoka, Kataoka Mayumi, Nakatsuji Hideaki, Tsutsumi Yutaka, Hashizume Atsushi, Adachi Hiroaki, Koike Haruki, Shinjo Keiko, Kondo Yutaka, Sobue Gen, Katsuno Masahisa

    EMBO MOLECULAR MEDICINE   Vol. 11 ( 5 )   2019.5

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    DOI: 10.15252/emmm.201708547

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  147. Efficacy and safety of leuprorelin acetate for subjects with spinal and bulbar muscular atrophy: pooled analyses of two randomized-controlled trials

    Hashizume Atsushi, Katsuno Masahisa, Suzuki Keisuke, Banno Haruhiko, Takeuchi Yu, Kawashima Motoshi, Suga Noriaki, Mano Tomoo, Araki Amane, Hijikata Yasuhiro, Hirakawa Akihiro, Sobue Gen, Sasaki H., Aoki M., Nakano I, Ito S., Mizusawa H., Yamamoto To, Hasegawa K., Miyajima H., Kanda N., Nakajima K., Tsujino A., Uchino M., Morita M., Kanai K.

    JOURNAL OF NEUROLOGY   Vol. 266 ( 5 ) page: 1211 - 1221   2019.5

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    DOI: 10.1007/s00415-019-09251-x

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  148. Long-term Effects of Androgen Deprivation in a Patient with Spinal and Bulbar Muscular Atrophy - A Case Report with 14 Years of Follow-up.

    Hijikata Y, Hashizume A, Yamada S, Ito D, Banno H, Suzuki K, Sobue G, Katsuno M

    Internal medicine (Tokyo, Japan)     2019.4

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.2169/internalmedicine.1592-18

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  149. Increase of arginine dimethylation correlates with the progression and prognosis of ALS.

    Ikenaka K, Atsuta N, Maeda Y, Hotta Y, Nakamura R, Kawai K, Yokoi D, Hirakawa A, Taniguchi A, Morita M, Mizoguchi K, Mochizuki H, Kimura K, Katsuno M, Sobue G

    Neurology   Vol. 92 ( 16 ) page: E1868 - E1877   2019.4

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    DOI: 10.1212/WNL.0000000000007311

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  150. Cardiac and peripheral vasomotor autonomic functions in hereditary transthyretin amyloidosis with non-Val30Met mutation

    Koike Haruki, Nakamura Tomohiko, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS   Vol. 26 ( sup1 ) page: 13 - 14   2019.3

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    DOI: 10.1080/13506129.2019.1582023

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  151. Evolution of amyloid fibrils in hereditary transthyretin amyloidosis: an ultrastructural study

    Koike Haruki, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS   Vol. 26 ( sup1 ) page: 26 - 26   2019.3

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    DOI: 10.1080/13506129.2019.1582496

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  152. Common clinicopathological features in late-onset hereditary transthyretin amyloidosis (Ala97Gly, Val94Gly and Val30Met)

    Koike Haruki, Nakamura Tomohiko, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Yasuda Takeshi, Mukai Eiichiro, Date Yukari, Shiomi Kazutaka, Nakazato Masamitsu, Katsuno Masahisa, Sobue Gen

    AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS   Vol. 26 ( sup1 ) page: 24 - 25   2019.3

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    DOI: 10.1080/13506129.2019.1582495

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  153. Cardiovascular autonomic functions in late-onset hereditary transthyretin amyloidosis with Val30Met mutation

    Nakamura Tomohiko, Koike Haruki, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS   Vol. 26 ( sup1 ) page: 6 - 6   2019.3

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    DOI: 10.1080/13506129.2019.1582018

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  154. Disease Affects Bdnf Expression in Synaptic and Extrasynaptic Regions of Skeletal Muscle of Three SBMA Mouse Models

    Halievski Katherine, Nath Samir R., Katsuno Masahisa, Adachi Hiroaki, Sobue Gen, Breedlove S. Marc, Lieberman Andrew P., Jordan Cynthia L.

    INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES   Vol. 20 ( 6 )   2019.3

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    DOI: 10.3390/ijms20061314

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  155. Ultrastructure in Transthyretin Amyloidosis: From Pathophysiology to Therapeutic Insights

    Koike Haruki, Katsuno Masahisa

    BIOMEDICINES   Vol. 7 ( 1 )   2019.2

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    DOI: 10.3390/biomedicines7010011

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  156. Four cases of multiple system atrophy with marked cortical atrophy and neuronal cytoplasmic inclusions in the temporal cortex

    Ando Takashi, Mimuro Maya, Iwasaki Yasushi, Katsuno Masahisa, Yoshida Mari

    BRAIN PATHOLOGY   Vol. 29   page: 84-85   2019.2

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  157. Five cases of cerebral amyloid angiopathy related inflammation/angiitis diagnosed with brain biopsy.

    Moriyoshi Hideyuki, Furukawa Soma, Ogura Aya, Ito Yasuhiro, Yoshida Mari, Katsuno Masahisa

    BRAIN PATHOLOGY   Vol. 29   page: 15-16   2019.2

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  158. New teased-fibre definitions represent specific mechanisms of neuropathy.

    Koike H, Katsuno M, Sobue G

    Journal of neurology, neurosurgery, and psychiatry   Vol. 90 ( 2 ) page: 124   2019.2

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    DOI: 10.1136/jnnp-2018-319493

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  159. New teased-fibre definitions represent specific mechanisms of neuropathy

    Koike Haruki, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY   Vol. 90 ( 2 ) page: 124-124   2019.2

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1136/jnnp-2018-319077

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  160. Vasculopathy in hereditary transthyretin amyloidosis: an electron microscopic study

    Koike Haruki, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Misumi Yohei, Ando Yukio, Ikeda Shu-ichi, Katsuno Masahisa, Sobue Gen

    BRAIN PATHOLOGY   Vol. 29   page: 29-29   2019.2

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  161. Ultrastructural mechanisms of macrophage-induced demyelination in chronic inflammatory demyelinating polyneuropathy: an observation on longitudinal sections

    Koike Haruki, Fukami Yuki, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    BRAIN PATHOLOGY   Vol. 29   page: 53-54   2019.2

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  162. Acute Unilateral Isolated Oculomotor Nerve Palsy in an Adult Patient with Influenza A.

    Senda J, Araki K, Tachi Y, Watanabe H, Kagawashira Y, Ito M, Atsuta N, Katsuno M, Watanabe H, Sobue G

    Internal medicine (Tokyo, Japan)   Vol. 58 ( 3 ) page: 433 - 436   2019

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    DOI: 10.2169/internalmedicine.0850-18

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  163. Hereditary Angioedema Type 1 with Recurrent Dizziness.

    Ando T, Torii R, Kazuta T, Endo T, Araki A, Horiuchi T, Terao S, Katsuno M

    Internal medicine (Tokyo, Japan)   Vol. 58 ( 13 ) page: 1961 - 1963   2019

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    DOI: 10.2169/internalmedicine.2015-18

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  164. Tau Accumulation and Network Breakdown in Alzheimer's Disease.

    Watanabe H, Bagarinao E, Yokoi T, Yamaguchi H, Ishigaki S, Mausuda M, Katsuno M, Sobue G

    Advances in experimental medicine and biology   Vol. 1184   page: 231 - 240   2019

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    DOI: 10.1007/978-981-32-9358-8_19

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  165. Ultrastructural mechanisms of macrophage-induced demyelination in CIDP

    Koike Haruki, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    NEUROLOGY   Vol. 91 ( 23 ) page: 1051 - 1060   2018.12

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    DOI: 10.1212/WNL.0000000000006625

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  166. Clinicopathological features of eosinophilic granulomatosis with polyangiitis with and without anti-neutrophil cytoplasmic antibodies

    Nishi Ryoji, Koike Haruki, Ikeda Shohei, Ohyama Ken, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 23 ( 4 ) page: 348-348   2018.12

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  167. IGF-1 for spinal and bulbar muscular atrophy: hope and challenges

    Hashizume Atsushi, Katsuno Masahisa

    LANCET NEUROLOGY   Vol. 17 ( 12 ) page: 1026 - 1027   2018.12

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    DOI: 10.1016/S1474-4422(18)30359-4

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  168. Evolution of amyloid fibrils in transthyretin familial amyloid polyneuropathy: An ultrastructural study

    Koike Haruki, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 23 ( 4 ) page: 321-321   2018.12

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  169. Ultrastructural mechanisms of macrophage-induced demyelination in chronic inflammatory demyelinating polyneuropathy

    Koike Haruki, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 23 ( 4 ) page: 364-365   2018.12

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  170. [Nonstenotic Carotid Plaque in Patients with Anterior Circulation Embolic Stroke of Undetermined Source].

    Hirunagi T, Miwa S, Katsuno M

    Brain and nerve = Shinkei kenkyu no shinpo   Vol. 70 ( 11 ) page: 1295-1299   2018.11

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    DOI: 10.11477/mf.1416201176

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  171. Involvement of the Precuneus/Posterior Cingulate Cortex Is Significant for the Development of Alzheimer's Disease: A PET (THK5351, PiB) and Resting fMRI Study

    Yokoi Takamasa, Watanabe Hirohisa, Yamaguchi Hiroshi, Bagarinao Epifanio, Masuda Michihito, Imai Kazunori, Ogura Aya, Ohdake Reiko, Kawabata Kazuya, Hara Kazuhiro, Riku Yuichi, Ishigaki Shinsuke, Katsuno Masahisa, Miyao Shinichi, Kato Katsuhiko, Naganawa Shinji, Harada Ryuichi, Okamura Nobuyuki, Yenai Kazuhiko, Yoshida Mari, Sobue Gen

    FRONTIERS IN AGING NEUROSCIENCE   Vol. 10   page: 304   2018.10

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    DOI: 10.3389/fnagi.2018.00304

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  172. The morphology of amyloid fibrils and their impact on tissue damage in hereditary transthyretin amyloidosis: An ultrastructural study.

    Koike H, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Sakurai T, Shimohata T, Katsuno M, Sobue G

    Journal of the neurological sciences   Vol. 394   page: 99-106   2018.9

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    DOI: 10.1016/j.jns.2018.09.011

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  173. Study protocol for the MEXiletine hydrochloride administration trial: a placebo-controlled, randomised, double-blind, multicentre, crossover study of its efficacy and safety in spinal and bulbar muscular atrophy (MEXPRESS).

    Yamada S, Hashizume A, Hijikata Y, Inagaki T, Ito D, Kinoshita F, Nakatochi M, Kobayashi Y, Hirakawa A, Nakamura T, Katsuno M

    BMJ open   Vol. 8 ( 9 ) page: e023041   2018.9

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    DOI: 10.1136/bmjopen-2018-023041

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  174. Clinical and Imaging Features of Multiple System Atrophy: Challenges for an Early and Clinically Definitive Diagnosis.

    Watanabe H, Riku Y, Hara K, Kawabata K, Nakamura T, Ito M, Hirayama M, Yoshida M, Katsuno M, Sobue G

    Journal of movement disorders   Vol. 11 ( 3 ) page: 107 - 120   2018.9

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    DOI: 10.14802/jmd.18020

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  175. Corpus callosal involvement is correlated with cognitive impairment in multiple system atrophy

    Hara Kazuhiro, Watanabe Hirohisa, Bagarinao Epifanio, Kawabata Kazuya, Yoneyama Noritaka, Ohdake Reiko, Imai Kazunori, Masuda Michihito, Yokoi Takamasa, Ogura Aya, Tsuboi Takashi, Ito Mizuki, Atsuta Naoki, Niwa Hisayoshi, Taoka Toshiaki, Maesawa Satoshi, Naganawa Shinji, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROLOGY   Vol. 265 ( 9 ) page: 2079 - 2087   2018.9

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    DOI: 10.1007/s00415-018-8923-7

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  176. Paraneoplastic autoimmune encephalitis associated with pleomorphic lung carcinoma: An autopsy case report

    Ando Takashi, Goto Yoji, Mano Kazuo, Nomura Fumio, Kurashige Masako, Ito Masafumi, Mimuro Maya, Iwasaki Yasushi, Katsuno Masahisa, Yoshida Mari

    NEUROPATHOLOGY   Vol. 38 ( 4 ) page: 448 - 454   2018.8

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    DOI: 10.1111/neup.12477

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  177. Preclinical progression of neurodegenerative diseases

    Katsuno Masahisa, Sahashi Kentaro, Iguchi Yohei, Hashizume Atsushi

    NAGOYA JOURNAL OF MEDICAL SCIENCE   Vol. 80 ( 3 ) page: 289 - 298   2018.8

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    DOI: 10.18999/nagjms.80.3.289

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  178. Pre-clinical symptoms of SBMA may not be androgen-dependent: implications from two SBMA mouse models

    Xu Youfen, Halievski Katherine, Katsuno Masahisa, Adachi Hiroaki, Sobue Gen, Breedlove S. Marc, Jordan Cynthia L.

    HUMAN MOLECULAR GENETICS   Vol. 27 ( 14 ) page: 2425 - 2442   2018.7

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    DOI: 10.1093/hmg/ddy142

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  179. Widespread Cardiac and Vasomotor Autonomic Dysfunction in Non-Val30Met Hereditary Transthyretin Amyloidosis.

    Koike H, Nakamura T, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Katsuno M, Sobue G

    Internal medicine (Tokyo, Japan)     2018.7

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    DOI: 10.2169/internalmedicine.1113-18

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  180. Systemic angiopathy and axonopathy in hereditary transthyretin amyloidosis with Ala97Gly (p. Ala117Gly) mutation: a post-mortem analysis.

    Koike H, Yasuda T, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Sobue G, Katsuno M

    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis     page: 1-2   2018.6

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    DOI: 10.1080/13506129.2018.1474734

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  181. Increased expression level of Hsp70 in the inner ears of mice by exposure to low frequency noise

    Ninomiya Hiromasa, Ohgami Nobutaka, Oshino Reina, Kato Masashi, Ohgami Kyoko, Li Xiang, Shen Dandan, Iida Machiko, Yajima Ichiro, Angelidis Charalampos E., Adachi Hiroaki, Katsuno Masahisa, Sobue Gen, Kato Masashi

    HEARING RESEARCH   Vol. 363   page: 49 - 54   2018.6

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    DOI: 10.1016/j.heares.2018.02.006

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  182. [Parkinson's disease associated with SAPHO syndrome: a case report].

    Ando T, Araki A, Terao S, Katsuno M

    Rinsho shinkeigaku = Clinical neurology   Vol. 58 ( 5 ) page: 332 - 334   2018.5

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    DOI: 10.5692/clinicalneurol.cn-001110

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  183. Silencing of FUS in the common marmoset (Callithrix jacchus) brain via stereotaxic injection of an adeno-associated virus encoding shRNA

    Endo Kuniyuki, Ishigaki Shinsuke, Masamizu Yoshito, Fujioka Yusuke, Watakabe Akiya, Yamamori Tetsuo, Hatanaka Nobuhiko, Nambu Atsushi, Okado Haruo, Katsuno Masahisa, Watanabe Hirohisa, Matsuzaki Masanori, Sobue Gen

    NEUROSCIENCE RESEARCH   Vol. 130   page: 56 - 64   2018.5

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    DOI: 10.1016/j.neures.2017.08.006

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  184. Biomarker-based analysis of preclinical progression in spinal and bulbar muscular atrophy

    Hijikata Yasuhiro, Hashizume Atsushi, Yamada Shinichiro, Inagaki Tomonori, Ito Daisuke, Hirakawa Akihiro, Suzuki Keisuke, Atsuta Naoki, Tsuboi Takashi, Hattori Makoto, Hori Akihiro, Banno Haruhiko, Sobue Gen, Katsuno Masahisa

    NEUROLOGY   Vol. 90 ( 17 ) page: E1501 - E1509   2018.4

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    DOI: 10.1212/WNL.0000000000005360

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  185. Frequency and characteristics of the TBK1 gene variants in Japanese patients with sporadic amyotrophic lateral sclerosis

    Tohnai Genki, Nakamura Ryoichi, Sone Jun, Nakatochi Masahiro, Yokoi Daichi, Katsuno Masahisa, Watanabe Hazuki, Watanabe Hirohisa, Ito Mizuki, Li Yuanzhe, Izumi Yuishin, Morita Mitsuya, Taniguchi Akira, Kano Osamu, Oda Masaya, Kuwabara Satoshi, Abe Koji, Aiba Ikuko, Okamoto Koichi, Mizoguchi Kouichi, Hasegawa Kazuko, Aoki Masashi, Hattori Nobutaka, Onodera Osamu, Naruse Hiroya, Mitsui Jun, Takahashi Yuji, Goto Jun, Ishiura Hiroyuki, Morishita Shinichi, Yoshimura Jun, Doi Koichiro, Tsuji Shoji, Nakashima Kenji, Kaji Ryuji, Atsuta Naoki, Sobue Gen

    NEUROBIOLOGY OF AGING   Vol. 64   page: 158.e15 - 158.e19   2018.4

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    DOI: 10.1016/j.neurobiolaging.2017.12.005

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  186. Distinct manifestation of cognitive deficits associate with different resting-state network disruptions in non-demented patients with Parkinson's disease

    Kawabata Kazuya, Watanabe Hirohisa, Hara Kazuhiro, Bagarinao Epifanio, Yoneyama Noritaka, Ogura Aya, Imai Kazunori, Masuda Michihito, Yokoi Takamasa, Ohdake Reiko, Tanaka Yasuhiro, Tsuboi Takashi, Nakamura Tomohiko, Hirayama Masaaki, Ito Mizuki, Atsuta Naoki, Maesawa Satoshi, Naganawa Shinji, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROLOGY   Vol. 265 ( 3 ) page: 688 - 700   2018.3

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    DOI: 10.1007/s00415-018-8755-5

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  187. Peritonitis after percutaneous endoscopic gastrojejunostomy for levodopa-carbidopa intestinal gel treatment despite concomitant use of gastropexy

    Tsuboi Takashi, Watanabe Hirohisa, Funasaka Kouhei, Takebayashi Mikiko, Miyata Kazushi, Katsuno Masahisa

    NEUROLOGY AND CLINICAL NEUROSCIENCE   Vol. 6 ( 2 ) page: 64-66   2018.3

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1111/ncn3.12183

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  188. Long-standing overt ventriculomegaly without aqueductal stenosis: A case report

    Ikumi Kazuhiro, Tsuboi Takashi, Atsuta Naoki, Takeuchi Kazuhito, Koike Haruki, Katsuno Masahisa

    NEUROLOGY AND CLINICAL NEUROSCIENCE   Vol. 6 ( 2 ) page: 42-44   2018.3

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1111/ncn3.12176

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  189. Treatment with Creatine Monohydrate in Spinal and Bulbar Muscular Atrophy: Protocol for a Randomized, Double-Blind, Placebo-Controlled Trial

    Hijikata Yasuhiro, Katsuno Masahisa, Suzuki Keisuke, Hashizume Atsushi, Araki Amane, Yamada Shinichiro, Inagaki Tomonori, Ito Daisuke, Hirakawa Akihiro, Kinoshita Fumie, Gosho Masahiko, Sobue Gen

    JMIR RESEARCH PROTOCOLS   Vol. 7 ( 3 ) page: e69   2018.3

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    DOI: 10.2196/resprot.8655

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  190. [New Diagnostic Criteria for Parkinson's Disease: MDS-PD Criteria].

    Watanabe H, Hara K, Ito M, Katsuno M, Sobue G

    Brain and nerve = Shinkei kenkyu no shinpo   Vol. 70 ( 2 ) page: 139 - 146   2018.2

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    DOI: 10.11477/mf.1416200966

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  191. Severe hyposmia and aberrant functional connectivity in cognitively normal Parkinson's disease. Reviewed

    Yoneyama N, Watanabe H, Kawabata K, Bagarinao E, Hara K, Tsuboi T, Tanaka Y, Ohdake R, Imai K, Masuda M, Hattori T, Ito M, Atsuta N, Nakamura T, Hirayama M, Maesawa S, Katsuno M, Sobue G.

    PLoS One.   Vol. 13 ( 1 ) page: e0190072   2018.1

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    DOI: 10.1371/journal.pone.0190072.

  192. Severe hyposmia and aberrant functional connectivity in cognitively normal Parkinson's disease

    Yoneyama Noritaka, Watanabe Hirohisa, Kawabata Kazuya, Bagarinao Epifanio, Hara Kazuhiro, Tsuboi Takashi, Tanaka Yasuhiro, Ohdake Reiko, Imai Kazunori, Masuda Michihito, Hattori Tatsuya, Ito Mizuki, Atsuta Naoki, Nakamura Tomohiko, Hirayama Masaaki, Maesawa Satoshi, Katsuno Masahisa, Sobue Gen

    PLOS ONE   Vol. 13 ( 1 ) page: e0190072   2018.1

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    DOI: 10.1371/journal.pone.0190072

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  193. Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years

    Koike Haruki, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Atsuta Naoki, Nakamura Tomohiko, Hirayama Masaaki, Ogata Hidenori, Yamasaki Ryo, Kira Jun-ichi, Katsuno Masahisa, Sobue Gen

    INTERNAL MEDICINE   Vol. 57 ( 14 ) page: 2061 - 2066   2018

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    DOI: 10.2169/internalmedicine.0455-17

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  194. Age-related impairment in Addenbrooke's cognitive examination revised scores in patients with amyotrophic lateral sclerosis

    Masuda Michihito, Watanabe Hirohisa, Tanaka Yasuhiro, Ohdake Reiko, Ogura Aya, Yokoi Takamasa, Imai Kazunori, Kawabata Kazuya, Riku Yuichi, Hara Kazuhiro, Nakamura Ryoichi, Atsuta Naoki, Katsuno Masahisa, Sobue Gen

    AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION   Vol. 19 ( 7-8 ) page: 578 - 584   2018

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    DOI: 10.1080/21678421.2018.1510009

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  195. Long-term treatment with leuprorelin for spinal and bulbar muscular atrophy: natural history-controlled study. Reviewed

    Hashizume A, Katsuno M, Suzuki K, Hirakawa A, Hijikata Y, Yamada S, Inagaki T, Banno H, Sobue G

    J Neurol Neurosurg Psychiatry.   Vol. 88 ( 12 ) page: 1026-1032   2017.12

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    DOI: 10.1136/jnnp-2017-316015

  196. Early detection of speech and voice disorders in Parkinson's disease patients treated with subthalamic nucleus deep brain stimulation: a 1-year follow-up study. Reviewed

    Tsuboi T, Watanabe H, Tanaka Y, Ohdake R, Hattori M, Kawabata K, Hara K, Ito M, Fujimoto Y, Nakatsubo D, Maesawa S, Kajita Y, Katsuno M, Sobue G.

    J Neural Transm (Vienna).   Vol. 124 ( 12 ) page: 1547-1556   2017.12

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    DOI: 10.1007/s00702-017-1804-x.

  197. Early detection of speech and voice disorders in Parkinson's disease patients treated with subthalamic nucleus deep brain stimulation: a 1-year follow-up study

    Tsuboi Takashi, Watanabe Hirohisa, Tanaka Yasuhiro, Ohdake Reiko, Hattori Makoto, Kawabata Kazuya, Hara Kazuhiro, Ito Mizuki, Fujimoto Yasushi, Nakatsubo Daisuke, Maesawa Satoshi, Kajita Yasukazu, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEURAL TRANSMISSION   Vol. 124 ( 12 ) page: 1547 - 1556   2017.12

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    DOI: 10.1007/s00702-017-1804-x

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  198. Cardiac and peripheral vasomotor autonomic functions in late-onset transthyretin Val30Met familial amyloid polyneuropathy. Reviewed

    Koike H, Nakamura T, Hashizume A, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Katsuno M, Sobue G.

    J Neurol.   Vol. 264 ( 11 ) page: 2293-2302   2017.11

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    DOI: 10.1007/s00415-017-8629-2.

  199. Structural MRI correlates of amyotrophic lateral sclerosis progression. Reviewed

    Senda J, Atsuta N, Watanabe H, Bagarinao E, Imai K, Yokoi D, Riku Y, Masuda M, Nakamura R, Watanabe H, Ito M, Katsuno M, Naganawa S, Sobue G.

      Vol. 88 ( 11 ) page: 901-907   2017.11

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    DOI: 10.1136/jnnp-2016-314337

  200. Cardiac and peripheral vasomotor autonomic functions in late-onset transthyretin Val30Met familial amyloid polyneuropathy

    Koike Haruki, Nakamura Tomohiko, Hashizume Atsushi, Nishi Ryoji, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROLOGY   Vol. 264 ( 11 ) page: 2293 - 2302   2017.11

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    DOI: 10.1007/s00415-017-8629-2

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  201. Distinct pathogenesis in nonsystemic vasculitic neuropathy and microscopic polyangiitis

    Takahashi Mie, Koike Haruki, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Hashizume Atsushi, Katsuno Masahisa, Sobue Gen

    NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION   Vol. 4 ( 6 ) page: e407   2017.11

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    DOI: 10.1212/NXI.0000000000000407

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  202. Non-motor multiple system atrophy associated with sudden death: pathological observations of autonomic nuclei

    Riku Yuichi, Watanabe Hirohisa, Mimuro Maya, Iwasaki Yasushi, Ito Mizuki, Katsuno Masahisa, Sobue Gen, Yoshida Mari

    JOURNAL OF NEUROLOGY   Vol. 264 ( 11 ) page: 2249 - 2257   2017.11

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    DOI: 10.1007/s00415-017-8604-y

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  203. Distinct pathogenesis in nonsystemic vasculitic neuropathy and microscopic polyangiitis. Reviewed

    Takahashi M, Koike H, Ikeda S, Kawagashira Y, Iijima M, Hashizume A, Katsuno M, Sobue G.

    Neurol Neuroimmunol Neuroinflamm.   Vol. 4 ( 6 ) page: e407   2017.10

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    DOI: 10.1212/NXI.0000000000000407.

  204. Highly asymmetric and subacutely progressive motor weakness with unilateral T2-weighted high intensities along the pyramidal tract in the brainstem in adrenomyeloneuropathy. Reviewed

    Tsuboi T, Tanaka Y, Yoshida Y, Nakamura T, Shimozawa N, Katsuno M.

    J Neurol Sci.   Vol. 381   page: 107-109   2017.10

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    DOI: 10.1016/j.jns.2017.08.018.

  205. Akt signaling pathway is dysregulated in polyglutamine diseases

    Iida M., Sahashi K., Kondo N., Nakatsuji H., Tohnai G., Tsutsumi Y., Adachi H., Sobue G., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 209-209   2017.10

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    DOI: 10.1016/j.jns.2017.08.597

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  206. Clinical presentation and diagnosis of neuronal intranuclear inclusion disease

    Sone J., Mori K., Haruki K., Nakamura T., Masuda M., Yoshida M., Iwasaki Y., Tanaka F., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 1017-1017   2017.10

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    DOI: 10.1016/j.jns.2017.08.2869

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  207. Clinical features of epilepsy patients on hemodialysis

    Araki K., Nakamura T., Horie K., Kobayashi Y., Kawakami O., Hamada K., Ando T., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 334-335   2017.10

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    DOI: 10.1016/j.jns.2017.08.949

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  208. Clinical characteristics of familial and sporadic amyotrophic lateral sclerosis patients with G93S mutation in the SOD1 gene

    Nakamura R., Atsuta N., Tohnai G., Yokoi D., Watanabe H., Hayashi N., Sone J., Ito M., Watanabe H., Katsuno M., Izumi Y., Hashimoto R., Aiba I., Mizoguchi K., Kaji R., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 568-568   2017.10

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    DOI: 10.1016/j.jns.2017.08.1599

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  209. Characteristics of semantic impairment in ALS associated with jukujikun

    Ogura A., Masuda M., Imai K., Ohdake R., Tanaka Y., Yokoi T., Kawabata K., Hara K., Nakamura R., Atsuta N., Watanabe H., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 571-571   2017.10

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    DOI: 10.1016/j.jns.2017.08.1607

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  210. Characteristics of cognitive features in ALS using the addenbrooke's cognitive examination revised (ACE-R)

    Masuda M., Imai K., Ohdake R., Tanaka Y., Ogura A., Yokoi T., Kawabata K., Hara K., Nakamura R., Atsuta N., Watanabe H., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 564-564   2017.10

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    DOI: 10.1016/j.jns.2017.08.1588

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  211. Association of impaired melatonin secretion with sleep disturbance in Parkinson's disease

    Nakamura T., Imai E., Ueda M., Suzuki M., Tohnai G., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 296-296   2017.10

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    DOI: 10.1016/j.jns.2017.08.842

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  212. Clinicopathological features of CIDP subtypes

    Ikeda S., Nishi R., Kawagashira Y., Iijima M., Koike H., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 465-466   2017.10

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    DOI: 10.1016/j.jns.2017.08.3520

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  213. The role of TDP-43 secretion in association with exosomes

    Iguchi Y., Eid L., Parent M., Riku Y., Kawai K., Yoshida M., Katsuno M., Sobue G., Julien J. P.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 208-209   2017.10

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    DOI: 10.1016/j.jns.2017.08.596

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  214. The retention pattern of THK5351 pet imaging and functional network disruption associated with development of Alzheimer's disease

    Yokoi T., Watanabe H., Yamaguchi H., Bagarinao E., Masuda M., Imai K., Ogura A., Ohdake R., Kawabata K., Hara K., Riku Y., Ishigaki S., Katsuno M., Kato K., Naganawa S., Okamura N., Miyao S., Yanai K., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 960-961   2017.10

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    DOI: 10.1016/j.jns.2017.08.2705

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  215. The impact of cold exposure in spinal and bulbar muscular atrophy

    Yamada S., Hashizume A., Hijikata Y., Inagaki T., Ito D., Nakamura T., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 1084-1084   2017.10

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    DOI: 10.1016/j.jns.2017.08.3058

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  216. The effect of noninvasive positive pressure ventilation in amyotrophic lateral sclerosis patients from a Japanese multicenter prospective cohort

    Yokoi D., Atsuta N., Hirakawa A., Nakamura R., Watanabe H., Hayashi N., Ito M., Watanabe H., Katsuno M., Izumi Y., Morita M., Taniguchi A., Oda M., Abe K., Mizoguchi K., Kano O., Kuwabara S., Kaji R., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 720-721   2017.10

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    DOI: 10.1016/j.jns.2017.08.2030

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  217. The analysis of neuromuscular pathology of spinal-bulbar muscular atrophy using iPSC-derived disease model

    Okada Y., Ito T., Tanaka S., Shimojo D., Onodera K., Katsuno M., Okano H., Sobue G., Doyu M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 572-572   2017.10

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    DOI: 10.1016/j.jns.2017.08.1611

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  218. Relationship between cardiac parasympathetic dysfunction and atrophy of the medulla oblongata in multiple system atrophy

    Suzuki M., Nakamura T., Hirayama M., Ueda M., Imai E., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 921-921   2017.10

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    DOI: 10.1016/j.jns.2017.08.2590

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  219. Reinnervation after denervation in inclusion body myositis

    Noda S., Koike H., Nakanishi H., Kimura S., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 825-826   2017.10

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    DOI: 10.1016/j.jns.2017.08.2323

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  220. Prognosis of japanese patients with amyotrophic lateral sclerosis according to motor phenotype

    Atsuta N., Yokoi D., Nakamura R., Watanabe H., Hayashi N., Ito M., Watanabe H., Katsuno M., Izumi Y., Morita M., Taniguchi A., Oda M., Abe K., Mizoguchi K., Kano O., Kuwabara S., Aoki M., Hattori N., Kaji R., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 103-103   2017.10

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    DOI: 10.1016/j.jns.2017.08.330

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  221. Preclinical biomarkers in spinal and bulbar muscular atrophy

    Hijikata Y., Hashizume A., Yamada S., Inagaki T., Ito D., Hirakawa A., Suzuki K., Banno H., Sobue G., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 462-462   2017.10

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    DOI: 10.1016/j.jns.2017.08.3512

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  222. Pathophysiological analysis of spinal and bulbar muscular atrophy using disease-specific iPSCs

    Onodera K., Simojo D., Ishihara Y., Doyu M., Okano H., Katsuno M., Sobue G., Okada Y.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 708-708   2017.10

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    DOI: 10.1016/j.jns.2017.08.1993

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  223. Paranodal axo-glial detachment in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies

    Koike H., Kadoya M., Kaida K. I., Nishi R., Ikeda S., Kawagashira Y., Lijima M., Kato D., Ogata H., Yamasaki R., Matsukawa N., Kira J. I., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 59 - 60   2017.10

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    DOI: 10.1016/j.jns.2017.08.226

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  224. Orthostatic hypotension in multiple system atrophy: Relation to symptoms and cognitive impairments

    Ueda M., Imai E., Suzuki M., Nakamura T., Hirayama M., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 922-922   2017.10

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    DOI: 10.1016/j.jns.2017.08.2593

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  225. Monozygotic twins concordant for autopsy-proven progressive supranuclear palsy

    Tsujikawa K., Sahashi K., Riku Y., Iwasaki Y., Yoshida M., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 1042-1043   2017.10

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    DOI: 10.1016/j.jns.2017.08.2944

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  226. Mediterranean fever gene mutations in patients with central nervous inflammation diagnosed with possible neuro-sweet disease

    Iijima M., Nishi R., Ikeda S., Kawagashira Y., Koike H., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 541-541   2017.10

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    DOI: 10.1016/j.jns.2017.08.3732

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  227. Longitudinal changes in speech and voice functions after subthalamic nucleus deep brain stimulation in Parkinson's disease patients

    Tsuboi T., Watanabe H., Tanaka Y., Ohdake R., Hattori M., Kawabata K., Hara K., Ito M., Nakatsubo D., Maesawa S., Kajita Y., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 1042-1042   2017.10

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    DOI: 10.1016/j.jns.2017.08.2943

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  228. Long-term treatment with leuprorelin for spinal and bulbar muscular atrophy

    Hashizume A., Katsuno M., Suzuki K., Hirakawa A., Hijikata Y., Yamada S., Inagaki T., Banno H., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 205-206   2017.10

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    DOI: 10.1016/j.jns.2017.08.588

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  229. Instability of syllable repetition in Parkinson's disease after subthalamic nucleus deep brain stimulation

    Tanaka Y., Tsuboi T., Watanabe H., Nakatsubo D., Maesawa S., Kajita Y., Hattori M., Ohdake R., Yamamoto M., Wakabatkabayashi T., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 1036-1036   2017.10

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    DOI: 10.1016/j.jns.2017.08.2924

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  230. HSV-2-Related hemophagocytic lymphohistiocytosis in an MS patient treated with fingolimod

    Ikumi K., Ando T., Katano H., Katsuno M., Sakai Y., Yoshida M., Saida T., Kimura H., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 441-441   2017.10

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    DOI: 10.1016/j.jns.2017.08.3457

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  231. Highly asymmetric and subacutely progressive motor weakness with unilateral T2-weighted high intensities along the pyramidal tract in the brainstem in adrenomyeloneuropathy

    Tsuboi Takashi, Tanaka Yasuhiro, Yoshida Yusuke, Nakamura Tomohiko, Shimozawa Nobuyuki, Katsuno Masahisa

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 107 - 109   2017.10

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    DOI: 10.1016/j.jns.2017.08.018

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  232. Estimation of skeletal muscle mass, serum creatinine and creatine kinase at the onset of amyotrophic lateral sclerosis

    Ito D., Hashizume A., Hijikata Y., Yamada S., Inagaki T., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 212-212   2017.10

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    DOI: 10.1016/j.jns.2017.08.606

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  233. Early cognitive decline pattern is associated with distinct resting-state networks disruption in non-demented Parkinson's disease patients

    Kawabata K., Watanabe H., Hara K., Bagarinao E., Yoneyama N., Ogura A., Imai K., Masuda M., Yokoi T., Ohdake R., Tsuboi T., Ito M., Atsuta N., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 128-128   2017.10

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    DOI: 10.1016/j.jns.2017.08.389

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  234. DNA methylation inhibitor mitigates spinal and bulbar muscular atrophy model mouse

    Kondo N., Sahashi K., Iida M., Nakatsuji H., Tohnai G., Adachi H., Sobue G., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 476-477   2017.10

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    DOI: 10.1016/j.jns.2017.08.3551

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  235. Diagnostic value of muscle biopsy for neuromuscular diseases

    Kimura S., Nakanishi H., Noda S., Murakami A., Iijima M., Koike H., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 473-474   2017.10

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    DOI: 10.1016/j.jns.2017.08.3543

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  236. Decision making alteration and characteristic connectivity changes in amyotrophic lateral sclerosis

    Imai K., Masuda M., Watanabe H., Kamagata K., Katahira K., Ogura A., Ohdake R., Kawabata K., Yokoi T., Hara K., Bagarinao E., Atsuta N., Nakamura R., Aoki S., Katsuno M., Ohira H., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 103-103   2017.10

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    DOI: 10.1016/j.jns.2017.08.331

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  237. Correlation between nerve conduction and progression rate patients with amyotrophic lateral sclerosis

    Imai E., Ueda M., Suzuki M., Nakamura T., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 492-492   2017.10

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    DOI: 10.1016/j.jns.2017.08.3593

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  238. Corpus callosum involvement by diffusion tensor imaging is early marker of cognitive decline in multiple system atrophy

    Hara K., Watanabe H., Bagarinao E., Kawabata K., Yoneyama N., Ohdake R., Imai K., Masuda M., Yokoi T., Tsuboi T., Ito M., Atsuta N., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 256-256   2017.10

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    DOI: 10.1016/j.jns.2017.08.730

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  239. Clinicopathological features of eosinophilic granulomatosis with polyangiitis with and without antineutrophil cytoplasmic antibodies

    Nishi R., Koike H., Ikeda S., Ohyama K., Kawagashira Y., Iijima M., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 825-825   2017.10

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    DOI: 10.1016/j.jns.2017.08.2322

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  240. The spatial accumulation patterns in normal elderly using 18F-THK5351 pet

    Yokoi T., Watanabe H., Aya O., Kawabata K., Imai K., Hara K., Masuda M., Ohdake R., Yamaguchi H., Miyao S. I., Katsuno M., Okamura N., Yanai K., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 960-960   2017.10

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    DOI: 10.1016/j.jns.2017.08.2704

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  241. Vasculopathy in familial amyloid polyneuropathy

    Koike H., Nishi R., Ikeda S., Kawagashira Y., Iijima M., Misumi Y., Ando Y., Ikeda S. I., Katsuno M., Sobue G.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 475-476   2017.10

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    DOI: 10.1016/j.jns.2017.08.3548

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  242. Understanding of the role of proteinaceous inclusions in spinal and bulbar muscular atrophy

    Sahashi K., Kondo N., Iida M., Nakatsuji H., Tohnai G., Iguchi Y., Sobue G., Katsuno M.

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 381   page: 709-709   2017.10

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    DOI: 10.1016/j.jns.2017.08.1997

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  243. 3'UTR Length-Dependent Control of SynGAP Isoform a2 mRNA by FUS and ELAV-like Proteins Promotes Dendritic Spine Maturation and Cognitive Function. Reviewed

    Yokoi S, Udagawa T, Fujioka Y, Honda D, Okado H, Watanabe H, Katsuno M, Ishigaki S, Sobue G.

    Cell Rep.   Vol. 20 ( 13 ) page: 3071-3084   2017.9

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    DOI: 10.1016/j.celrep.2017.08.100.

  244. 3 ' UTR Length-Dependent Control of SynGAP Isoform alpha 2 mRNA by FUS and ELAV-like Proteins Promotes Dendritic Spine Maturation and Cognitive Function

    Yokoi Satoshi, Udagawa Tsuyoshi, Fujioka Yusuke, Honda Daiyu, Okado Haruo, Watanabe Hirohisa, Katsuno Masahisa, Ishigaki Shinsuke, Sobue Gen

    CELL REPORTS   Vol. 20 ( 13 ) page: 3071 - 3084   2017.9

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    DOI: 10.1016/j.celrep.2017.08.100

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  245. Pathologic Involvement of Glutamatergic Striatal Inputs From the Cortices in TAR DNA-Binding Protein 43 Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis Reviewed

    Riku Y, Watanabe H, Yoshida M, Mimuro M, Iwasaki Y, Masuda M, Ishigaki S, Katsuno M, Sobue G.

    J Neuropathol Exp Neurol.   Vol. 76 ( 9 ) page: 759-768   2017.9

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  246. CLINICOPATHOLOGICAL FEATURES AMONG CIDP SUBTYPES

    Ikeda S., Nishi R., Kawagashira Y., Iijima M., Koike H., Katsuno M., Sobue G.

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 22 ( 3 ) page: 303-304   2017.9

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  247. SCHWANN CELL AND ENDOTHELIAL CELL DAMAGE IN TRANSTHYRETIN FAMILIAL AMYLOID POLYNEUROPATHY

    Koike H., Ikeda S., Takahashi M., Kawagashira Y., Iijima M., Misumi Y., Ando Y., Ikeda S. I., Katsuno M., Sobue G.

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 22 ( 3 ) page: 318-318   2017.9

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  248. Prevalence of anti-NT5C1A antibodies in Japanese patients with autoimmune rheumatic diseases in comparison with other patient cohorts

    Muro Yoshinao, Nakanishi Hirotaka, Katsuno Masahisa, Kono Michihiro, Akiyama Masashi

    CLINICA CHIMICA ACTA   Vol. 472   page: 1 - 4   2017.9

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    DOI: 10.1016/j.cca.2017.07.002

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  249. Pathologic Involvement of Glutamatergic Striatal Inputs From the Cortices in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

    Riku Yuichi, Watanabe Hirohisa, Yoshida Mari, Mimuro Maya, Iwasaki Yasushi, Masuda Michihito, Ishigaki Shinsuke, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY   Vol. 76 ( 9 ) page: 759 - 768   2017.9

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    DOI: 10.1093/jnen/nlx055

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  250. PARANODAL DISSECTION IN CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY WITH ANTI-NEUROFASCIN 155 AND ANTI-CONTACTIN 1 ANTIBODIES

    Koike H., Kadoya M., Kaida K., Ikeda S., Kawagashira Y., Iijima M., Kato D., Ogata H., Yamasaki R., Matsukawa N., Kira J. I, Katsuno M., Sobue G.

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 22 ( 3 ) page: 318-318   2017.9

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  251. EFFICACY OF IMMUNOGLOBULINS FOR NOD B7-2 KO MICE

    Iijima M., Nishi R., Ikeda S., Kawagashira Y., Koike H., Sobue G., Katsuno M.

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   Vol. 22 ( 3 ) page: 303-303   2017.9

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  252. Reply: Neuronal intranuclear (hyaline) inclusion disease and fragile X-associated tremor/ataxia syndrome: a morphological and molecular dilemma

    Sone Jun, Nakamura Tomohiko, Koike Haruki, Katsuno Masahisa, Tanaka Fumiaki, Iwasaki Yasushi, Yoshida Mari, Sobue Gen

    BRAIN   Vol. 140 ( 8 ) page: E52 - +   2017.8

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    DOI: 10.1093/brain/awx158

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  253. Swallowing markers in spinal and bulbar muscular atrophy

    Banno Haruhiko, Katsuno Masahisa, Suzuki Keisuke, Tanaka Seiya, Suga Noriaki, Hashizume Atsushi, Mano Tomoo, Araki Amane, Watanabe Hirohisa, Fujimoto Yasushi, Yamamoto Masahiko, Sobue Gen

    ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY   Vol. 4 ( 8 ) page: 534 - 543   2017.8

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    DOI: 10.1002/acn3.425

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  254. Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies. Reviewed

    Koike H, Kadoya M, Kaida KI, Ikeda S, Kawagashira Y, Iijima M, Kato D, Ogata H, Yamasaki R, Matsukawa N, Kira JI, Katsuno M, Sobue G.

    J Neurol Neurosurg Psychiatry.   Vol. 88 ( 6 ) page: 465-473   2017.6

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    DOI: 10.1136/jnnp-2016-314895.

  255. Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies

    Koike Haruki, Kadoya Masato, Kaida Ken-ichi, Ikeda Shohei, Kawagashira Yuichi, Iijima Masahiro, Kato Daisuke, Ogata Hidenori, Yamasaki Ryo, Matsukawa Noriyuki, Kira Jun-ichi, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY   Vol. 88 ( 6 ) page: 465 - 473   2017.6

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    DOI: 10.1136/jnnp-2016-314895

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  256. Correlation of insulin resistance and motor function in spinal and bulbar muscular atrophy. Reviewed

    Nakatsuji H, Araki A, Hashizume A, Hijikata Y, Yamada S, Inagaki T, Suzuki K, Banno H, Suga N, Okada Y, Ohyama M, Nakagawa T, Kishida K, Funahashi T, Shimomura I, Okano H, Katsuno M, Sobue G.

    J Neurol.   Vol. 264 ( 5 ) page: 839-847   2017.5

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  257. Correlation of insulin resistance and motor function in spinal and bulbar muscular atrophy

    Nakatsuji Hideaki, Araki Amane, Hashizume Atsushi, Hijikata Yasuhiro, Yamada Shinichiro, Inagaki Tomonori, Suzuki Keisuke, Banno Haruhiko, Suga Noriaki, Okada Yohei, Ohyama Manabu, Nakagawa Tohru, Kishida Ken, Funahashi Tohru, Shimomura Iichiro, Okano Hideyuki, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROLOGY   Vol. 264 ( 5 ) page: 839 - 847   2017.5

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    DOI: 10.1007/s00415-017-8405-3

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  258. TRANSFORMING GROWTH FACTOR-beta SIGNALING IS UPREGULATED IN SPORADIC INCLUSION BODY MYOSITIS

    Noda Seiya, Koike Haruki, Maeshima Shinya, Nakanishi Hirotaka, Iijima Masahiro, Matsuo Koji, Kimura Seigo, Katsuno Masahisa, Sobue Gen

    MUSCLE & NERVE   Vol. 55 ( 5 ) page: 741 - 747   2017.5

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    DOI: 10.1002/mus.25405

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  259. Diagnostic flowchart of adult-onset neuronal intranuclear inclusion disease

    Sone Jun, Mori Keiko, Koike Haruki, Nakamura Tomohiko, Masuda Michihito, Yoshida Mari, Iwasaki Yasushi, Tanaka Fumiaki, Katsuno Masahisa, Sobue Gen

    NEUROLOGY   Vol. 88   2017.4

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  260. Acute disseminated encephalomyelitis in China, Singapore, and Japan: a comparison with the USA

    Koelman Diederik, Benkeser David, Xu Yan, Neo Shermyn, Tan Kevin, Katsuno Masahisa, Sobue Gen, Natsume Jun, Chahin Salim, Mar Soe, Venkatesan Arun, Chitnis Tanuja, Hoganson George, Yeshokumar Anusha, Barreras Paula, Majmudar Bittu, Carone Marco, Mateen Farrah

    NEUROLOGY   Vol. 88   2017.4

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  261. Disruption of blood-nerve barriers in hereditary transthyretin (ATTR) amyloidosis

    Koike Haruki, Ikeda Shohei, Takahashi Mie, Kawagashira Yuichi, Iijima Masahiro, Misumi Yohei, Ando Yukio, Ikeda Shu-ichi, Katsuno Masahisa, Sobue Gen

    AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS   Vol. 24 ( sup1 ) page: 89 - 90   2017.3

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    DOI: 10.1080/13506129.2017.1278691

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  262. Efficacy of a novel drug, zonisamide, in the modulation of oxidative stress in Parkinson's disease

    Hirayama Masaaki, Minato Tomomi, Maeda Tetsuya, Nakamura Tomohiko, Katsuno Masahisa

    NEUROLOGY AND CLINICAL NEUROSCIENCE   Vol. 5 ( 2 ) page: 49-54   2017.3

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1111/ncn3.12103

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  263. Acute disseminated encephalomyelitis in China, Singapore and Japan: a comparison with the USA. Reviewed

    Koelman DL, Benkeser DC, Xu Y, Neo SX, Tan K, Katsuno M, Sobue G, Natsume J, Chahin S, Mar SS, Venkatesan A, Chitnis T, Hoganson GM, Yeshokumar AK, Barreras P, Majmudar B, Carone M, Mateen FJ.

    Eur J Neurol.   Vol. 24 ( 2 ) page: 391-396.   2017.2

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    DOI: doi: 10.1111/ene.13220.

  264. Acute disseminated encephalomyelitis in China, Singapore and Japan: a comparison with the USA

    Koelman D. L. H., Benkeser D. C., Xu Y., Neo S. X., Tan K., Katsuno M., Sobue G., Natsume J., Chahin S., Mar S. S., Venkatesan A., Chitnis T., Hoganson G. M., Yeshokumar A. K., Barreras P., Majmudar B., Carone M., Mateen F. J.

    EUROPEAN JOURNAL OF NEUROLOGY   Vol. 24 ( 2 ) page: 391-396   2017.2

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    DOI: 10.1111/ene.13220

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  265. Cardiac parasympathetic dysfunction in the early phase of Parkinson's disease

    Suzuki Masashi, Nakamura Tomohiko, Hirayama Masaaki, Ueda Miki, Katsuno Masahisa, Sobue Gen

    JOURNAL OF NEUROLOGY   Vol. 264 ( 2 ) page: 333 - 340   2017.2

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    DOI: 10.1007/s00415-016-8348-0

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  266. Altered Tau Isoform Ratio Caused by Loss of FUS and SFPQ Function Leads to FTLD-like Phenotypes. Reviewed

    Ishigaki S, Fujioka Y, Okada Y, Riku Y, Udagawa T, Honda D, Yokoi S, Endo K, Ikenaka K, Takagi S, Iguchi Y, Sahara N, Takashima A, Okano H, Yoshida M, Warita H, Aoki M, Watanabe H, Okado H, Katsuno M, Sobue G.

    Cell Rep.   Vol. 18 ( 5 ) page: 1118-1131.   2017.1

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    DOI: doi: 10.1016/j.celrep.2017.01.013.

  267. Altered Tau Isoform Ratio Caused by Loss of FUS and SFPQ Function Leads to FTLD-like Phenotypes. Reviewed

    Ishigaki S, Fujioka Y, Okada Y, Riku Y, Udagawa T, Honda D, Yokoi S, Endo K, Ikenaka K, Takagi S, Iguchi Y, Sahara N, Takashima A, Okano H, Yoshida M, Warita H, Aoki M, Watanabe H, Okado H, Katsuno M, Sobue G.

      Vol. 18 ( 5 ) page: 1118-1131.   2017.1

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  268. Altered Tau Isoform Ratio Caused by Loss of FUS and SFPQ Function Leads to FTLD-like Phenotypes

    Ishigaki Shinsuke, Fujioka Yusuke, Okada Yohei, Riku Yuichi, Udagawa Tsuyoshi, Honda Daiyu, Yokoi Satoshi, Endo Kuniyuki, Ikenaka Kensuke, Takagi Shinnosuke, Iguchi Yohei, Sahara Naruhiko, Takashima Akihiko, Okano Hideyuki, Yoshida Mari, Warita Hitoshi, Aoki Masashi, Watanabe Hirohisa, Okado Haruo, Katsuno Masahisa, Sobue Gen

    CELL REPORTS   Vol. 18 ( 5 ) page: 1118 - 1131   2017.1

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    DOI: 10.1016/j.celrep.2017.01.013

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  269. Lower body mass index is associated with orthostatic hypotension in Parkinson's disease

    Nakamura Tomohiko, Suzuki Masashi, Ueda Miki, Hirayama Masaaki, Katsuno Masahisa

    JOURNAL OF THE NEUROLOGICAL SCIENCES   Vol. 372   page: 14-18   2017.1

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    DOI: 10.1016/jjns.2016.11.027

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  270. Lower body mass index is associated with orthostatic hypotension in Parkinson's disease.

    Nakamura T, Suzuki M, Ueda M, Hirayama M, Katsuno M

    Journal of the neurological sciences   Vol. 372   page: 14-18   2017.1

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    DOI: 10.1016/j.jns.2016.11.027

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  271. Alcoholic Myelopathy and Nutritional Deficiency

    Koike Haruki, Nakamura Tomohiko, Ikeda Shohei, Takahashi Mie, Kawagashira Yuichi, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    INTERNAL MEDICINE   Vol. 56 ( 1 ) page: 105 - 108   2017

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    DOI: 10.2169/internalmedicine.56.7364

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  272. Progress toward the development of treatment of spinal and bulbar muscular atrophy

    Sahashi Kentaro, Hashizume Atsushi, Sobue Gen, Katsuno Masahisa

    EXPERT OPINION ON ORPHAN DRUGS   Vol. 5 ( 6 ) page: 503-514   2017

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1080/21678707.2017.1329088

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  273. Quantitative Assessment of Swallowing Dysfunction in Patients with Spinal and Bulbar Muscular Atrophy

    Hashizume Atsushi, Banno Haruhiko, Katsuno Masahisa, Hijikata Yasuhiro, Yamada Shinichiro, Inagaki Tomonori, Suzuki Keisuke, Sobue Gen

    INTERNAL MEDICINE   Vol. 56 ( 23 ) page: 3159 - 3165   2017

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    DOI: 10.2169/internalmedicine.8799-16

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  274. Vasculitic Neuropathy Following Exposure to a Glyphosate-based Herbicide

    Kawagashira Yuichi, Koike Haruki, Kawabata Kazuya, Takahashi Mie, Ohyama Ken, Hashimoto Rina, Iijima Masahiro, Katsuno Masahisa, Sobue Gen

    INTERNAL MEDICINE   Vol. 56 ( 11 ) page: 1431 - 1434   2017

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    DOI: 10.2169/internalmedicine.56.8064

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  275. Decreased Peak Expiratory Flow Associated with Muscle Fiber-Type Switching in Spinal and Bulbar Muscular Atrophy. Reviewed

    Yamada S, Hashizume A, Hijikata Y, Inagaki T, Suzuki K, Kondo N, Kawai K, Noda S, Nakanishi H, Banno H, Hirakawa A, Koike H, Halievski K, Jordan CL, Katsuno M, Sobue G.

    PLoS One.   Vol. 11 ( 12 ) page: e0168846.   2016.12

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    DOI: doi: 10.1371/journal.pone.0168846.

  276. Exosome secretion is a key pathway for clearance of pathological TDP-43. Reviewed

    Iguchi Y, Eid L, Parent M, Soucy G, Bareil C, Riku Y, Kawai K, Takagi S, Yoshida M, Katsuno M, Sobue G, Julien JP.

    Brain   Vol. 139 ( 12 ) page: 3187-3201   2016.12

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  277. Clinicopathological features of adult-onset neuronal intranuclear inclusion disease. Reviewed

    Sone J, Mori K, Inagaki T, Katsumata R, Takagi S, Yokoi S, Araki K, Kato T, Nakamura T, Koike H, Takashima H, Hashiguchi A, Kohno Y, Kurashige T, Kuriyama M, Takiyama Y, Tsuchiya M, Kitagawa N, Kawamoto M, Yoshimura H, Suto Y, Nakayasu H, Uehara N, Sugiyama H, Takahashi M, Kokubun N, Konno T, Katsuno M, Tanaka F, Iwasaki Y, Yoshida M, Sobue G.

    Brain   Vol. 139 ( 12 ) page: 3170-3186.   2016.12

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  278. Next-generation sequencing of 28 ALS-related genes in a Japanese ALS cohort. Reviewed

    Nakamura R, Sone J, Atsuta N, Tohnai G, Watanabe H, Yokoi D, Nakatochi M, Watanabe H, Ito M, Senda J, Katsuno M, Tanaka F, Li Y, Izumi Y, Morita M, Taniguchi A, Kano O, Oda M, Kuwabara S, Abe K, Aiba I, Okamoto K, Mizoguchi K, Hasegawa K, Aoki M, Hattori N, Tsuji S, Nakashima K, Kaji R, Sobue G; Japanese Consortium for Amyotrophic Lateral Sclerosis Research (JaCALS).

    Neurobiol Aging.   Vol. 39 ( 219 ) page: e1-8   2016

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    DOI: doi: 10.1016/j.neurobiolaging.2015.11.030.

  279. Association of Leukoaraiosis With Convalescent Rehabilitation Outcome in Patients With Ischemic Stroke. Reviewed

    Senda J, Ito K, Kotake T, Kanamori M, Kishimoto H, Kadono I, Suzuki Y, Katsuno M, Nishida Y, Ishiguro N, Sobue G.

    Stroke   Vol. 47 ( 1 ) page: 160-166   2016

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    DOI: doi: 10.1161/STROKEAHA.115.010682.

  280. Pioglitazone suppresses neuronal and muscular degeneration caused by polyglutamine-expanded androgen receptors. Reviewed

    Iida M, Katsuno M, Nakatsuji H, Adachi H, Kondo N, Miyazaki Y, Tohnai G, Ikenaka K, Watanabe H, Yamamoto M, Kishida K, Sobue G.

    Hum Mol Genet.   Vol. 24 ( 2 ) page: 314-329   2015

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    DOI: 10.1093/hmg/ddu445

  281. Head Lift Exercise Improves Swallowing Dysfunction in Spinal and Bulbar Muscular Atrophy. Reviewed

    Mano T, Katsuno M, Banno H, Suzuki K, Suga N, Hashizume A, Araki A, Hijikata Y, Tanaka S, Takatsu J, Watanabe H, Yamamoto M, Sobue G.

    Eur Neurol.   Vol. 74 ( 5-6 ) page: 251-258   2015

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  282. Silencing neuronal mutant androgen receptor in a mouse model of spinal and bulbar muscular atrophy. Reviewed

    Sahashi K, Katsuno M, Hung G, Adachi H, Kondo N, Nakatsuji H, Tohnai G, Iida M, Bennett CF, Sobue G.

    Hum Mol Genet.   Vol. 24 ( 21 ) page: 5985-5994   2015

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  283. FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilization. Reviewed

    Udagawa T, Fujioka Y, Tanaka M, Honda D, Yokoi S, Riku Y, Ibi D, Nagai T, Yamada K, Watanabe H, Katsuno M, Inada T, Ohno K, Sokabe M, Okado H, Ishigaki S, Sobue G.

    Nat Commun.   Vol. 6   page: 7098   2015

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1038/ncomms8098

  284. Astrocyte-Derived TGF-β1 Accelerates Disease Progression in ALS Mice by Interfering with the Neuroprotective Functions of Microglia and T Cells. Reviewed

    Endo F, Komine O, Fujimori-Tonou N, Katsuno M, Jin S, Watanabe S, Sobue G, Dezawa M, Wyss-Coray T, Yamanaka K.

      Vol. 11 ( 4 ) page: 592-604   2015

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    DOI: 10.1016/j.celrep.2015.03.053

  285. Tongue pressure as a novel biomarker of spinal and bulbar muscular atrophy (SBMA). Reviewed

    Mano T, Katsuno M, Banno H, Suzuki K, Suga N, Hashizume A, Araki A, Watanabe H, Tanaka S, Yamamoto M, Sobue G

      Vol. 82   page: 255-262   2014

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  286. Brugada syndrome in spinal and bulbar muscular atrophy. Reviewed

    Araki A, Katsuno M, Suzuki K, Banno H, Suga N, Hashizume A, Mano T, Hijikata Y, Nakatsuji H, Watanabe H, Yamamoto M, Makiyama T, Ohno S, Fukuyama M, Morimoto S, Horie M, Sobue G.

    Neurology   Vol. 82   page: 1813-1821   2014

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  287. Paeoniflorin eliminates a mutant AR via NF-YA-dependent proteolysis in spinal and bulbar muscular atrophy. Reviewed

    Tohnai G, Adachi H, Katsuno M, Doi H, Matsumoto S, Kondo N, Miyatzaki Y, Iida M, Nakatsuji H, Qiang Q, Ding Y, Watanabe H, Yamamoo M, Ohtsuka K, Sobue G.

    Hum Mol Genet   Vol. 23   page: 3552-3565   2014

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  288. Schwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3. Reviewed

    Suga N, Katsuno M, Koike H, Banno H, Suzuki K, Hashizume A, Mano T, Iijima M, Kawagashira Y, Hirayama M, Nakamura T, Watanabe H, Tanaka F, Sobue G.

    Neuropathol Appl Neurobiol.   Vol. 40   page: 628-639   2014

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    DOI: 10.1111/nan.12055

  289. Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration. Reviewed

    Kondo N, Katsuno M, Adachi H, Minamiyama M, Doi H, Matsumoto S, Miyazaki Y, Iida M, Tohnai G, Nakatsuji H, Ishigaki S, Fujioka Y, Watanabe H, Tanaka F, Nakai A, Sobue G.

    Nat Commun   Vol. 4   page: 1405   2013

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  290. Cortical and subcortical brain atrophy in Parkinson's disease with visual hallucination. Reviewed

    Watanabe H, Senda J, Kato S, Ito M, Atsuta N, Hara K, Tsuboi T, Katsuno M, Nakamura T, Hirayama M, Adachi H, Naganawa S, Sobue G.

    Mov Disord   Vol. 28   page: 1732-1736   2013

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    DOI: 10.1002/mds.25641

  291. Amyotrophic lateral sclerosis: an update on recent genetic insights. Invited Reviewed

    Iguchi Y, Katsuno M, Ikenaka K, Ishigaki S, Sobue G

    J Neurol   Vol. 260   page: 2917-2927   2013

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1007/s00415-013-7112-y.

  292. FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD. Reviewed

    Fujioka Y, Ishigaki S, Masuda A, Iguchi Y, Udagawa T, Watanabe H, Katsuno M, Ohno K, Sobue G

    Sci Rep   Vol. 3   page: 2388   2013

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    DOI: 10.1038/srep02388

  293. RNP2 of RNA Recognition Motif 1 Plays a Central Role in the Aberrant Modification of TDP-43. Reviewed

    Takagi S, Iguchi Y, Katsuno M, Ishigaki S, Ikenaka K, Fujioka Y, Honda D, Niwa J, Tanaka F, Watanabe H, Adachi H, Sobue G

      Vol. 8   page: e66966   2013

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1371/journal.pone.0066966

  294. A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases. Reviewed

    Fujita K, Nakamura Y, Oka T, Ito H, Tamura T, Tagawa K, Sasabe T, Katsuta A, Motoki K, Shiwaku H, Sone M, Yoshida C, Katsuno M, Eishi Y, Murata M, Paul Taylor J, Wanker EE, Kono K, Tashiro S, Sobue G, La Spada AR, Okazawa H

      Vol. 4   page: 1816   2013

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1038/ncomms2828

  295. p62/SQSTM1 Differentially Removes the Toxic Mutant Androgen Receptor via Autophagy and Inclusion Formation in a Spinal and Bulbar Muscular Atrophy Mouse Model. Reviewed

    Doi H, Adachi H, Katsuno M, Minamiyama M, Matsumoto S, Kondo N, Miyazaki Y, Iida M, Tohnai G, Qiang Q, Tanaka F, Yanagawa T, Warabi E, Ishii T, Sobue G

      Vol. 33   page: 7710-7727   2013

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1523/JNEUROSCI.3021-12.2013

  296. Loss of TDP-43 causes age-dependent progressive motor neuron degeneration. Reviewed

    Iguchi Y, Katsuno M, Niwa JI, Takagi S, Ishigaki S, Ikenaka K, Kawai K, Watanabe H, Yamanaka K, Takahashi R, Misawa H, Sasaki S, Tanaka F, Sobue G

    Brain   Vol. 136   page: 1371-1382   2013

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    Language:English   Publishing type:Research paper (scientific journal)  

    DOI: 10.1093/brain/awt029

  297. Translational research on disease-modifying therapies for neurodegenerative diseases. Invited Reviewed

    Katsuno M, Watanabe H, Tanaka F, Sobue G

    Neurology and Clinical Neuroscience   Vol. 1   page: 3-10   2013

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    Authorship:Lead author   Language:English  

    DOI: 10.1002/ncn3.7

  298. dnc-1/dynactin 1 knockdown disrupts transport of autophagosomes and induces motor neuron degeneration. Reviewed

    Ikenaka K, Kawai K, Katsuno M, Huang Z, Jiang YM, Iguchi Y, Kobayashi K, Kimata T, Waza M, Tanaka F, Mori I, Sobue G

    PLoS One   Vol. 8   page: e54511   2013

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    DOI: 10.1371/journal.pone.0054511

  299. Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions. Reviewed

    Ishigaki S, Masuda A, Fujioka Y, Iguchi Y, Katsuno M, Shibata A, Urano F, Sobue G, Ohno K.

    Sci Rep.   Vol. 2   page: 529   2012

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  300. Molecular pathophysiology and disease-Modifying therapies for spinal and bulbar muscular atrophy. Invited Reviewed

    Katsuno M, Banno H, Suzuki K, Adachi H, Tanaka F, Sobue G.

    Arch Neurol.   Vol. 69   page: 436-440   2012

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  301. Differential change of clinical outcome measures in spinal and bulbar muscular atrophy: comparison of natural history with placebo-treated group. Reviewed

    Hashizume A, Katsuno M, Banno H, Suzuki K, Suga N, Tanaka F, Sobue G.

    J Neurol.   Vol. 259   page: 712-719   2012

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  302. Perspectives on molecular targeted therapies and clinical trials for neurodegenerative diseases. Invited Reviewed

    Katsuno M, Tanaka F, Sobue G.

    J Neurol Neurosurg Psychiatry   Vol. 83   page: 329-335   2012

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  303. Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies. Reviewed

    Iguchi Y, Katsuno M, Takagi S, Ishigaki S, Niwa J-I, Hasegawa M, Tanaka F, Sobue G.

    Neurobiol Dis.   Vol. 45   page: 862-870   2012

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  304. Viral delivery of miR-196a ameliorates the SBMA phenotype via the silencing of CELF2. Reviewed

    Miyazaki Y, Adachi H, Katsuno M, MInamiyama M, Jiang YM, Huang Z, Doi H, Matsumoto S, Kondo N, Iida M, Tohnai G, Tanaka F, Muramatsu S, Sobue G.

    Nat Med.   Vol. 18   page: 1136-1141   2012

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  305. c-Abl inhibition delays motor neuron degeneration in the G93A mouse, an animal model of amyotrophic lateral sclerosis. Reviewed

    Katsumata R, Ishigaki S, Katsuno M, Kawai K, Sone J, Huang Z, Adachi H, Tanaka F, Urano F, Sobue G.

    PLoS One   Vol. 7   page: e46185   2012

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  306. Naratriptan mitigates CGRP1-associated motor neuron degeneration caused by expanded polyglutamine. Reviewed

    Minamiyama M, Katsuno M, Adachi H, Doi H, Kondo N, Iida M, Ishigaki S, Fujioka Y, Matsumoto S, Miyazaki Y, Tanaka F, Kurihara H, Sobue G.

    Nat Med.   Vol. 18   page: 1531-1538   2012

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  307. Longitudinal changes of outcome measures in spinal and bulbar muscular atrophy. Reviewed

    Hashizume A, Katsuno M, Banno H, Suzuki K, Suga N, Mano T, Atsuta N, Oe H, Watanabe H, Tanaka F, Sobue G.

    Brain   Vol. 135   page: 2838-2848   2012

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  308. TSUNAMI: an antisense method to phenocopy splicing-associated diseases in animals.

    Sahashi K, Hua Y, Ling KK, Hung G, Rigo F, Horev G, Katsuno M, Sobue G, Ko CP, Bennett CF, Krainer AR.

    Genes Dev.   Vol. 26   page: 1874-84   2012

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  309. Cross-sectional and longitudinal analysis of an oxidative stress biomarker for spinal and bulbar muscular atrophy (SBMA). Reviewed

    Mano T, Katsuno M, Banno H, Suzuki K, Suga N, Hashizume A, Tanaka F, Sobue G.

    Muscle Nerve   Vol. 46   page: 692-697   2012

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  310. Macroautophagy Is Regulated by the UPR-Mediator CHOP and Accentuates the Phenotype of SBMA Mice. Reviewed

    Yu Z, Wang AM, Adachi H, Katsuno M, Sobue G, Yue Z, Robins DM, Lieberman AP.

    PLoS Genet.   Vol. 7   page: e1002321   2011

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  311. Dutasteride for spinal and bulbar muscular atrophy: another advance towards disease-modifying therapy. Invited Reviewed

    Banno H, Katsuno M, Suzuki K, Sobue G.

    Lancet Neurol.   Vol. 10   page: 113-115   2011

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  312. Transforming growth factor-beta signaling in motor neuron diseases. Invited Reviewed

    Katsuno M, Adachi H, Banno H, Suzuki K, Tanaka F, Sobue G.

    Current Mol Med.   Vol. 11   page: 48-56   2011

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  313. The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy. Reviewed

    Suzuki K, Katsuno M, Banno H, Takeuchi Y, Kawashima M, Suga N, Hashizume A, Hama T, Uchida K, Yamashita F, Nakamura T, Hirayama M, Tanaka F, Sobue G.

    J Neurol Neurosurg Psychiatry   Vol. 81   page: 567-571   2010

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  314. Clinical features and molecular mechanisms of spinal and bulbar muscular atrophy (SBMA). Invited Reviewed

    Katsuno M, Banno H, Suzuki K, Adachi H, Tanaka F, Sobue G.

    Adv Exp Med Biol.   Vol. 685   page: 64-74   2010

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  315. Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study): a multicentre, randomised, double-blind, placebo-controlled trial. Reviewed

    Katsuno M, Banno H, Suzuki K, Takeuchi Y, Kawashima M, Yabe I, Sasaki H, Aoki M, Morita M, Nakano I, Kanai K, Ito S, Ishikawa K, Mizusawa H, Yamamoto T, Tsuji S, Hasegawa K, Shimohata T, Nishizawa M, Miyajima H, Kanda F, Watanabe Y, Nakashima K, Tsujino A, Yamashita T, Uchino M, Fujimoto Y, Tanaka F, Sobue G; for the Japan SBMA Interventional Trial for TAP-144-SR (JASMITT) study group.

    Lancet Neurol.   Vol. 9   page: 875-884   2010

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  316. Disrupted TGF-beta signaling in spinal and bulbar muscular atrophy. Reviewed

    Katsuno M, Adachi H, Minamiyama M, Waza M, Doi H, Kondo N, Mizoguchi H, Nitta A, Yamada K, Banno H, Suzuki K, Tanaka F, Sobue G.

    J Neurosci.   Vol. 30   page: 5702-5712   2010

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  317. 17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in a SBMA model mouse. Reviewed

    Tokui K, Adachi H, Waza M, Katsuno M, Minamiyama M, Doi H, Tanaka K, Hamazaki J, Murata S, Tanaka F, Sobue G.

    Hum Mol Genet   Vol. 18   page: 898-910   2009

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  318. Heat shock proteins in neurodegenerative diseases: Pathogenic roles and therapeutic implications. Reviewed

    Adachi H, Katsuno M, Waza M, Minamiyama M, Tanaka F, Sobue G.

    Int J Hyperthermia   Vol. 25   page: 647-654   2009

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  319. Correlation between pyramidal tract degeneration and widespread white matter involvement in amyotrophic lateral sclerosis: a study with tractography and diffusion-tensor imaging. Reviewed

    Senda J, Ito M, Watanabe H, Atsuta N, Kawai Y, Katsuno M, Tanaka F, Naganawa S, Fukatsu H, Sobue G.

    Amyotroph Lateral Scler.   Vol. 10   page: 288-294   2009

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  320. Single nucleotide polymorphism of TAG-1 influences IVIg responsiveness of Japanese patients with CIDP. Reviewed

    Iijima M, Tomita M, Morozumi S, Kawagashira Y, Nakamura T, Koike H, Katsuno M, Hattori N, Tanaka F, Yamamoto M, Sobue G.

    Neurology   Vol. 73   page: 1348-1352   2009

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  321. Distinct characteristics of amyloid deposits in early- and late-onset transthyretin Val30Met familial amyloid polyneuropathy. Reviewed

    Koike H, Ando Y, Ueda M, Kawagashira Y, Iijima M, Fujitake J, Hayashi M, Yamamoto M, Mukai E, Nakamura T, Katsuno M, Hattori N, Sobue G.

    J Neurol Sci.   Vol. 287   page: 178-184   2009

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  322. Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy. Reviewed

    Palazzolo I, Stack C, Kong L, Musaro A, Adachi H, Katsuno M, Sobue G, Taylor JP, Sumner CJ, Fischbeck KH, Pennuto M.

    Neuron   Vol. 63   page: 316-328   2009

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  323. TDP-43 Depletion Induces Neuronal Cell Damage through Dysregulation of Rho Family GTPases. Reviewed

    Iguchi Y, Katsuno M, Niwa J, Yamada S, Sone J, Waza M, Adachi H, Tanaka F, Nagata K, Arimura N, Watanabe T, Kaibuchi K, Sobue G.

    J Biol Chem.   Vol. 284   page: 2059-22066   2009

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  324. Getting a handle on Huntington's disease: the case for cholesterol. Invited

    Katsuno M*, Adachi H, Sobue G.

    Nat Med.   Vol. 15   page: 253-254   2009

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  325. Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy. Reviewed

    Banno H, Katsuno M*, Suzuki K, Takeuchi Y, Kawashima M, Suga N, Takamori M, Ito M, Nakamura T, Matsuo K, Yamada S, Oki Y, Adachi H, Minamiyama M, Waza M, Atsuta N, Watanabe H, Fujimoto Y, Nakashima T, Tanaka F, Doyu M, Sobue G.

    Ann Neurol.   Vol. 65   page: 140-150   2009

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  326. B-type natriuretic peptide and cardiovalvulopathy in Parkinson disease with dopamine agonist. Reviewed

    Watanabe H, Hirayama M, Noda A, Ito M, Atsuta N, Senda J, Kaga T, Yamada A, Katsuno M, Niwa T, Tanaka F, Sobue G.

    Neurology   Vol. 72   page: 621-626   2009

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  327. Correlation between pyramidal tract degeneration and widespread white matter involvement in amyotrophic lateral sclerosis: A study with tractography and diffusion-tensor imaging. Reviewed

    Senda J, Ito M, Watanabe H, Atsuta N, Kawai Y, Katsuno M, Tanaka F, Naganawa S, Fukatsu H, Sobue G.

    Amyotroph Lateral Scler.   Vol. 29   page: 1-8   2009

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  328. Neuropathology and therapeutic intervention in spinal and bulbar muscular atrophy. Invited

    Banno H, Katsuno M*, Suzuki K, Tanaka F. Sobue G.

      Vol. 10   page: 1000-1012   2009

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  329. Pathogenesis-targeting therapeutics for spinal and bulbar muscular atrophy (SBMA). Invited

    Suzuki K, Katsuno M, Banno H, Sobue G.

    Neuropathology   Vol. 29   page: 509-516   2009

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  330. Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sj&ouml;gren's syndrome. Reviewed

    Morozumi S, Kawagashira Y, Iijima M, Koike H, Hattori N, Katsuno M, Tanaka F, Sobue G.

    J Neurol Sci.   Vol. 279   page: 57-61   2009

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  331. Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy. Reviewed

    Banno H, Katsuno M, Suzuki K, Takeuchi Y, Kawashima M, Suga N, Takamori M, Ito M, Nakamura T, Matsuo K, Yamada S, Oki Y, Adachi H, Minamiyama M, Waza M, Atsuta N, Watanabe H, Fujimoto Y, Nakashima T, Tanaka F, Doyu M, *Sobue G.

    Ann Neurol.   Vol. 65   page: 140-150   2009

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  332. Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy. Reviewed

    Palazzolo I, Stack C, Kong L, Musaro A, Adachi H, Katsuno M, Sobue G, Taylor JP, Sumner CJ, Fischbeck KH, Pennuto M.

    Neuron   Vol. 63   page: 316-328   2009

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  333. TDP-43 Depletion Induces Neuronal Cell Damage through Dysregulation of Rho Family GTPases. Reviewed

    Iguchi Y, Katsuno M, Niwa J, Yamada S, Sone J, Waza M, Adachi H, Tanaka F, Nagata K, Arimura N, Watanabe T, Kaibuchi K, Sobue G.

    J Biol Chem.   Vol. 284   page: 22059-22066   2009

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  334. Getting a handle on Huntington's disease: the case for cholesterol. Invited Reviewed

    Katsuno M, Adachi H, *Sobue G.

    Nat Med.   Vol. 15   page: 253-254   2009

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  335. Walking capacity evaluated by the 6-minute walk test in spinal and bulbar muscular atrophy. Reviewed

    Takeuchi Y, Katsuno M, Banno H, Suzuki K, Kawashima M, Atsuta N, Ito M, Watanabe H, Tanaka F, Sobue G.

    muscle Nerve   Vol. 38 ( 2 ) page: 964-971   2008.8

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  336. Cognitive impairment in spinocerebellar ataxia type 6. Reviewed

    Suenaga M, Kawai Y, Watanabe H, Atsuta N, Ito M, Tanaka F, Katsuno M, Fukatsu H, Naganawa S, Sobue G.

    J Neurol Neurosurg Psychiatry   Vol. 79   page: 496-499   2008

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  337. Walking capacity evaluated by the 6-minute walk test in spinal and bulbar muscular atrophy. Reviewed

    Takeuchi Y, Katsuno M*, Banno H, Suzuki K, Kawashima M, Atsuta N, Ito M, Watanabe H, Tanaka F, Sobue G.

    Muscle Nerve   Vol. 38   page: 964-971   2008

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  338. Prevalence and incidence rates of chronic inflammatory demyelinating polyneuropathy in the Japanese population Reviewed

    Iijima M, Koike H, Hattori N, Tamakoshi A, Katsuno M, Tanaka F, Yamamoto M, Arimura K, Sobue G.

    J Neurol Neurosurg Psychiatry   Vol. 79   page: 1040-1043   2008

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  339. Molecular Genetics and Biomarkers of Polyglutamine Diseases. Reviewed

    Katsuno M, Banno H, Suzuki K, Takeuchi Y, Kawashima M, Adachi H, Tanaka F, Sobue G

    Current Mol Med   Vol. 8   page: 221-234   2008

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  340. Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy. Invited Reviewed

    Adachi H, Waza M, Katsuno M, Tanaka F, Doyu M, Sobue G. Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy.

    Neuropathol Appl Neurobiol   Vol. 33 ( 2 ) page: 135-151   2007

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  341. CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA. Reviewed

    6. Suzuki K, Katsuno M, Banno H, Takeuchi Y, Atsuta N, Ito M, Watanabe H, Yamashita F, Hori N, Nakamura T, Hirayama M, Tanaka F, Sobue G.

    Brain   Vol. 131   page: 229-239   2007

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  342. Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A. Reviewed

    7. Koike H, Iijima M, Mori K, Yamamoto M, Hattori N, Katsuno M, Tanaka F, Watanabe H, Doyu M, Yoshikawa H, Sobue G.

    J Neuropathol Exp Neurol   Vol. 66   page: 1027-1036   2007

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  343. Therapeutic strategies for spinal and bulbar muscular atrophy (SBMA). Invited

    Katsuno M, Banno H, Suzuki K, Takeuchi Y, Minamiyama M, Waza M, Adachi H, Tanaka F, Sobue G.

    Drug Future   Vol. 32   page: 907-917   2007

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  344. Gene expressions specifically detected in motor neurons (dynactin 1, early growth response 3, acetyl-CoA transporter, death receptor 5, and cyclin C) differentially correlate to pathologic markers in sporadic amyotrophic lateral sclerosis. Reviewed

      Vol. 66   page: 617-627   2007

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  345. Disulfide Bond Mediates Aggregation, Toxicity, and Ubiquitylation of Familial Amyotrophic Lateral Sclerosis-linked Mutant SOD1. Reviewed

    Niwa J, Yamada S, Ishigaki S, Sone J, Takahashi M, Katsuno M, Tanaka F, Doyu M, Sobue G.

    J Biol Chem   Vol. 282 ( 38 ) page: 28087-28095   2007

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  346. Intravenous immunoglobulin therapy markedly ameliorates muscle weakness and severe pain in proximal diabetic neuropathy. Reviewed

    Kawagashira Y, Watanabe H, Oki Y, Iijima M, Koike H, Hattori N, Katsuno M, Tanaka F, Sobue G.

    J Neurol Neurosurg Psychiatry   Vol. 78 ( 8 ) page: 899-901   2007

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  347. CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model. Reviewed

    Adachi H, Waza M, Tokui K, Katsuno M, Minamiyama M, Tanaka F, Doyu M, Sobue G.

    J Neurosci   Vol. 27 ( 19 ) page: 5115-5126   2007

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  348. Ataxic vs painful form of paraneoplastic neuropathy. Reviewed

    Oki Y, Koike H, Iijima M, Mori K, Hattori N, Katsuno M, Nakamura T, Hirayama M, Tanaka F, Shiraishi M, Yazaki S, Nokura K, Yamamoto H, Sobue G.

    Neurology   Vol. 69 ( 6 ) page: 564-572   2007

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  349. Modulation of Hsp90 function in neurodegenerative disorders: a molecular-targeted therapy against disease-causing protein. Invited Reviewed

    Waza M, Adachi H, Katsuno M, Minamiyama M, Tanaka F, Doyu M, Sobue G.

    J Mol Med   Vol. 84   page: 635-646   2006

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  350. Alleviating Neurodegeneration by an Anticancer Agent: An Hsp90 Inhibitor (17-AAG). Invited Reviewed

    Waza M, Adachi H, Katsuno M, Minamiyama M, Tanaka F, Sobue G.

    Ann N Y Acad Sci   Vol. 1086   page: 21-34   2006

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  351. Reversible disruption of dynactin 1-mediated retrograde axonal transport in polyglutamine-induced motor neuron degeneration. Reviewed

    Katsuno M, Adachi H, Minamiyama M, Waza M, Tokui K, Banno H, Suzuki K, Onoda Y, Tanaka F, Doyu M, Sobue G.

    J Neurosci   Vol. 26   page: 12106-12117   2006

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    Spinal and bulbar muscular atrophy (SBMA) is a hereditary neurodegenerative disease caused by an expansion of a trinucleotide CAG repeat encoding the polyglutamine tract in the androgen receptor (AR) gene. To elucidate the pathogenesis of polyglutamine-mediated motor neuron dysfunction, we investigated histopathological and biological alterations in a transgenic mouse model of SBMA carrying human pathogenic AR. In affected mice, neurofilaments and synaptophysin accumulated at the distal motor axon. A similar intramuscular accumulation of neurofilament was detected in the skeletal muscle of SBMA patients. Fluoro-gold labeling and sciatic nerve ligation demonstrated an impaired retrograde axonal transport in the transgenic mice. The mRNA level of dynactin 1, an axon motor for retrograde transport, was significantly reduced in the SBMA mice resulting from pathogenic AR-induced transcriptional dysregulation. These pathological events were observed before the onset of neurological symptoms, but were reversed by castration, which prevents nuclear accumulation of pathogenic AR. Overexpression of dynactin 1 mitigated neuronal toxicity of the pathogenic AR in a cell culture model of SBMA. These observations indicate that polyglutamine-dependent transcriptional dysregulation of dynactin 1 plays a crucial role in the reversible neuronal dysfunction in the early stage of SBMA.

  352. Gene Expression Profiling toward Understanding of ALS Pathogenesis. Invited Reviewed

    Tanaka F, Niwa J, Ishigaki S, Katsuno M, Waza M, Yamamoto M, Doyu M, Sobue G.

    Ann N Y Acad Sci   Vol. 1086   page: 1-10   2006

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  353. Mutant androgen receptor accumulation in spinal and bulbar muscular atrophy scrotal skin: a pathogenic marker. Reviewed

    Banno H, Adachi H, Katsuno M, Suzuki K, Atsuta N, Watanabe H, Tanaka F, Doyu M, Sobue G.

    Ann Neurol   Vol. 59   page: 520-526   2006

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  354. Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients. Reviewed

    Atsuta N, Watanabe H, Ito M, Banno H, Suzuki K, Katsuno M, Tanaka F, Tamakoshi A, Sobue G.

    Brain   Vol. 129   page: 1446-1455   2006

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  355. Pathogenesis, animal models and therapeutics in Spinal and Bulbar Muscular Atrophy (SBMA). Invited Reviewed

    Katsuno M, Adachi H, Waza M, Banno H, Suzuki K, Tanaka F, Doyu M, Sobue G.

    Exp Neurol   Vol. 200   page: 8-18   2006

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  356. Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: A comparative study. Reviewed

    Sugiura M, Koike H, Iijima M, Mori K, Hattori N, Katsuno M, Tanaka F, Sobue G.

    J Neurol Sci   Vol. 241   page: 31-37   2006

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  357. Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients. Reviewed

    Adachi H, Katsuno M, Minamiyama M, Waza M, Sang C, Nakagomi Y, Kobayashi Y, Tanaka F, Doyu M, Inukai A, Yoshida M, Hashizume Y, Sobue G.

    Brain   Vol. 128   page: 659-670   2005

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  358. Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Reviewed

    Katsuno M, Sang C, Adachi H, Minamiyama M, Waza M, Tanaka F, Doyu M, Sobue G.

    Proc Natl Acad Sci USA   Vol. 102   page: 16801-16806   2005

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    Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motor neuron disease caused by the expansion of a trinucleotide CAG repeat encoding the polyglutamine tract in the first exon of the androgen receptor gene (AR). The pathogenic, polyglutamine-expanded AR protein accumulates in the cell nucleus in a ligand-dependent manner and inhibits transcription by interfering with transcriptional factors and coactivators. Heat-shock proteins (HSPs) are stress-induced chaperones that facilitate the refolding and, thus, the degradation of abnormal proteins. Geranylgeranylacetone (GGA), a nontoxic antiulcer drug, has been shown to potently induce HSP expression in various tissues, including the central nervous system. In a cell model of SBMA, GGA increased the levels of Hsp70, Hsp90, and Hsp105 and inhibited cell death and the accumulation of pathogenic AR. Oral administration of GGA also up-regulated the expression of HSPs in the central nervous system of SBMA-transgenic mice and suppressed nuclear accumulation of the pathogenic AR protein, resulting in amelioration of polyglutamine-dependent neuromuscular phenotypes. These observations suggest that, although a high dose appears to be needed for clinical effects, oral GGA administration is a safe and promising therapeutic candidate for polyglutamine-mediated neurodegenerative diseases, including SBMA.

  359. 17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Reviewed

    Waza M, Adachi H, Katsuno M, Minamiyama M, Sang C, Tanaka F, Inukai A, Doyu M, Sobue G.

    Nat Med   Vol. 11   page: 1088-1095   2005

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    Heat-shock protein 90 (Hsp90) functions as part of a multichaperone complex that folds, activates and assembles its client proteins. Androgen receptor (AR), a pathogenic gene product in spinal and bulbar muscular atrophy (SBMA), is one of the Hsp90 client proteins. We examined the therapeutic effects of 17-allylamino-17-demethoxygeldanamycin (17-AAG), a potent Hsp90 inhibitor, and its ability to degrade polyglutamine-expanded mutant AR. Administration of 17-AAG markedly ameliorated motor impairments in the SBMA transgenic mouse model without detectable toxicity, by reducing amounts of monomeric and aggregated mutant AR. The mutant AR showed a higher affinity for Hsp90-p23 and preferentially formed an Hsp90 chaperone complex as compared to wild-type AR; mutant AR was preferentially degraded in the presence of 17-AAG in both cells and transgenic mice as compared to wild-type AR. 17-AAG also mildly induced Hsp70 and Hsp40. 17-AAG would thus provide a new therapeutic approach to SBMA and probably to other related neurodegenerative diseases.

  360. The wide spectrum of clinical manifestations in Sjogren's syndrome-associated neuropathy. Reviewed

    Mori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H, Katsuno M, Fujita A, Aiba I, Ogata A, Saito T, Asakura K, Yoshida M, Hirayama M, Sobue G.

    Brain   Vol. 128   page: 2518-2534   2005

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  361. Geranylgeranylacetone ameliorates ischemic acute renal failure via induction of Hsp70. Reviewed

    Suzuki S, Maruyama S, Sato W, Morita Y, Sato F, Miki Y, Kato S, Katsuno M, Sobue G, Yuzawa Y, Matsuo S.

    Kidney Int   Vol. 67   page: 2210-2220   2005

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    Language:English   Publishing type:Research paper (scientific journal)  

  362. Clinical and electrophysiologic correlates of IVIg responsiveness in CIDP. Reviewed

    Iijima M, Yamamoto M, Hirayama M, Tanaka F, Katsuno M, Mori K, Koike H, Hattori N, Arimura K, Nakagawa M, Yoshikawa H, Hayasaka K, Onodera O, Baba M, Yasuda H, Saito T, Nakazato M, Nakashima K, Kira J, Kaji R, Oka N, Sobue G.

    Neurology   Vol. 64   page: 1471-1475   2005

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    Language:English   Publishing type:Research paper (scientific journal)  

  363. Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis. Reviewed

    Jiang YM, Yamamoto M, Kobayashi Y, Yoshihara T, Liang Y, Terao S, Takeuchi H, Ishigaki S, Katsuno M, Adachi H, Niwa J, Tanaka F, Doyu M, Yoshida M, Hashizume Y, Sobue G.

    Ann Neurol   Vol. 57   page: 236-251   2005

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  364. Multiple regional 1H-MR spectroscopy in multiple system atrophy: NAA/Cr reduction in pontine base as a valuable diagnostic marker. Reviewed

    Watanabe H, Fukatsu H, Katsuno M, Sugiura M, Hamada K, Okada Y, Hirayama M, Ishigaki T, Sobue G.

    J Neurol Neurosurg Psychiatry   Vol. 75   page: 103-109   2004

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  365. Polyglutamine diminishes VEGF; passage to motor neuron death? Invited Reviewed

    Katsuno M, Sobue G.

    Neuron   Vol. 41   page: 677-679   2004

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    Authorship:Lead author   Language:English  

  366. Sweet relief for Huntington disease. Invited Reviewed

    Katsuno M, Adachi H, Sobue G.

    Nat Med   Vol. 10   page: 123-124   2004

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    Authorship:Lead author   Language:English  

  367. Spinal and bulbar muscular atrophy: ligand-dependent pathogenesis and therapeutic perspectives. Invited Reviewed

    Katsuno M, Adachi H, Tanaka F, Sobue G.

    J Mol Med   Vol. 82   page: 298-307   2004

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  368. Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Reviewed

    Minamiyama M, Katsuno M, Adachi H, Waza M, Sang C, Kobayashi Y, Tanaka F, Doyu M, Inukai A, Sobue G.

    Hum Mol Genet   Vol. 13   page: 1183-92   2004

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    Spinal and bulbar muscular atrophy (SBMA) is an inherited motor neuron disease caused by the expansion of a polyglutamine (polyQ) tract within the androgen receptor. Unifying mechanisms have been implicated in the pathogenesis of polyQ-dependent neurodegenerative diseases including SBMA, Huntington disease and spinocerebellar ataxias. It has been suggested that mutant protein containing polyQ inhibits histone acetyltransferase activity, resulting in transcriptional dysfunction and subsequent neuronal dysfunction. Histone deacetylase (HDAC) inhibitors alleviate neurological phenotypes in fly and mouse models of polyQ disease, although the therapeutic effect is limited by the toxicity of these compounds. We studied the therapeutic effects of sodium butyrate (SB), an HDAC inhibitor, in a transgenic mouse model of SBMA. Oral administration of SB ameliorated neurological phenotypes as well as increased acetylation of nuclear histone in neural tissues. These therapeutic effects, however, were seen only within a narrow range of SB dosage. Our results indicate that SB is a possible therapeutic agent for SBMA and other polyQ diseases, although an appropriate dose should be determined for clinical application.

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Research Project for Joint Research, Competitive Funding, etc. 3

  1. 球脊髄性筋萎縮症の適正治療に関するエビデンス構築のための臨床研究

    2018.4 - 2021.3

    難治性疾患克服研究事業  

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    Grant type:Competitive

  2. 球脊髄性筋萎縮症の神経筋システム変性を標的とした革新的治療法開発

    2017.4 - 2020.3

    難治性疾患克服研究事業 

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    Grant type:Competitive

  3. ポリグルタミン病の包括的治療開発

    2009.4 - 2015.3

    CREST 

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    Grant type:Competitive

    球脊髄性筋萎縮症における細胞内シグナルの異常の解明とそれに基づく治療法開発

KAKENHI (Grants-in-Aid for Scientific Research) 26

  1. 運動ニューロン疾患におけるニューロサーキット変性の病態解明と治療法開発

    2010.4 - 2015.3

    科学研究費補助金  新学術領域研究,課題番号:22110005

    勝野 雅央

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    Authorship:Principal investigator 

  2. 球脊髄性筋萎縮症における活動依存性シナプス病態の解明と治療法の開発

    2012.4 - 2014.3

    科学研究費補助金 

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    Authorship:Principal investigator 

    球脊髄性筋萎縮症モデルマウスの神経筋接合部における分子変化を網羅的に解析し、治療法開発へと繋げる。

  3. 運動ニューロン疾患における神経・筋システム変性の分子病態解明と治療法開発

    2014.4 - 2017.3

    科学研究費補助金  基盤研究(B)

    勝野 雅央

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    運動ニューロン疾患は根本治療法のない進行性かつ致命的神経変性疾患である。本研究では運動ニューロン疾患の病態を単なるニューロン変性としてではなく、神経・筋システム変性として捉え、ALS・SBMAの複数のモデルマウス・患者iPS由来神経・筋共培養系・患者剖検組織・バイオマーカーなどのマテリアルを駆使し、神経・筋システム変性に寄与する分子変化を同定し、運動ニューロン・骨格筋クロストークの異常を標的とした治療法を開発する。研究期間に、(1)運動ニューロン疾患における神経・筋システム変性を惹起する細胞内シグナル、(2)Cre-loxP-STOPマウスによる神経・筋クロストーク異常に関わる細胞間シグナル、(3)マウスモデルにおける運動療法の分子メカニズムと患者における運動療法の有効性、(4)NMJにおける分子変化をターゲットした治療法の有効性、を明らかにすることを目的とする。

  4. 球脊髄性筋萎縮症におけるクレアチニン代謝異常の病態解明と探索的臨床試験

    2014.4 - 2015.3

    科学研究費補助金 

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    Authorship:Principal investigator 

    球脊髄性筋萎縮症(SBMA)はアンドロゲン受容体遺伝子変異を原因とする運動ニューロン疾患であるが、近年その病態に骨格筋障害が関与していることが示唆されている。申請者らはこれまでの研究においてSBMA患者では他の神経筋疾患の患者に比べて、骨格筋量に比して血清Cr値が低下していることを見出しており、本疾患では骨格筋におけるCr代謝が阻害されていることが示唆される。本研究はこうした知見に基づき、疾患モデルおよび患者組織を用いてSBMA骨格筋におけるCr代謝異常の分子機構を解明し、SBMA患者に対するクレチン補充療法の臨床試験(フェーズIIa)を実施し、安全性・用量反応性および有効性について解析し、新規の病態機構の解明と対症療法の開発を進める。

  5. 運動ニューロン疾患における神経・筋システム変性の分子病態解明と治療法開発

    2017.4 - 2020.3

    科学研究費補助金  基盤研究(B)

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    Authorship:Principal investigator 

  6. Wide-field Ca imaging and trans-omics for mouse model of Lewy body disease

    Grant number:21K19443  2021.7 - 2023.3

    Grants-in-Aid for Scientific Research  Grant-in-Aid for Challenging Research (Exploratory)

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    Authorship:Principal investigator 

    Grant amount:\6500000 ( Direct Cost: \5000000 、 Indirect Cost:\1500000 )

  7. 球脊髄性筋萎縮症レジストリデータを用いた病態進行に関わる因子の同定

    Grant number:21K07458  2021.4 - 2024.3

    科学研究費助成事業  基盤研究(C)

    橋詰 淳, 勝野 雅央, 山田 晋一郎

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    2017年8月、リュープロレリン酢酸塩に「球脊髄性筋萎縮症(SBMA)の進行抑制」の効能が追加された。実臨床下での有効性・安全性プロファイルをより確実に構築するためには、広く生体試料も含め収集された臨床データを解析し、予後規定因子の同定をさらに進めることが重要である。そのためには、prodromal期の患者や女性保因者も含めたデータベースを構築し、各種バイオマーカーについて、一気通貫に解析することが必要である。本研究は、名古屋大学神経内科が既に構築しているSBMA患者レジストリをさらに発展的に構築し、網羅的に収集した臨床データ、各種バイオマーカーを利用し、その予後規定因子を同定する。

  8. 孤発性ALSにおける凝集型TDP-43の生体内伝播メカニズムの解明

    Grant number:20H03589  2020.4 - 2023.3

    科学研究費助成事業  基盤研究(B)

    井口 洋平, 佐橋 健太郎, 勝野 雅央

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    孤発性筋萎縮性側索硬化症において凝集型TDP-43がニューロン間をプリオンのように伝播することが想定されるが、実際に生体内でこのTDP-43のプリオン様伝播を証明した事例はない。研究代表者らはCre発現マウスとAAV-CMV-FLEXベクターを用いることによりTDP-43の生体内における細胞間伝播を強く示唆する結果を得ている。本研究では凝集型TDP-43の生体内伝播を”確実に”証明し、次にその伝播を修飾する因子を検討しsALSの病態抑止療法への展開を目指す。
    代表者らはCre発現マウスとAAV-CMV-FLEXベクターを用いることによりTDP-43の生体内における細胞間伝播の検証を行った。CamKII-Creマウス一側海馬にAAV-CMV-FLEX-TDPmNLS-GFPベクターを投与し4週後に病理を観察すると同側海馬を中心としてGFPの発現が見られたが、視床前核や扁桃体、嗅内野、対側の海馬ニューロンにもGFPの発現が確認された。CreレポーターマウスによるCamKII-CreマウスにおけるCre発現パターン解析やin situ ハイブリダイゼーション法によるGFPmRNA発現解析から、TDP-GFPの遠隔領域の発現はAAVの順行性/逆行性活性ではなく、注入側海馬からの細胞間伝播によるものと考えられた。AAV-CMV-FLEX-TDPmNLSmRRM-GFPを注射したマウスでは遠隔領域のニューロンにおけるGFPの発現も確認されたがmNLS型に比し乏しかった。しかし注入側海馬近傍の脳梁ではオリゴデンドロサイト(OLG)内にGFPの発現が観察され、OLGでのTDP-GFPの発現はニューロン-OLG(N-O)間伝播と考えられた。より凝集性の強い変異であるTDPmNLSmRRMは遠隔への伝播は困難であるがN-O間に伝播しやすい可能性が示唆された。また、一側運動野の上位運動ニューロンに発現させたTDP-GFPのシグナルが2週間後にin vivo imagingで対側運動ニューロンで確認することができた。さらに、CamKII-Creマウスの一次運動野とChAT-Creマウスの脊髄前角にAAV-CMV-FLEX-TDPmNLS-GFPを注入して運動ニューロン間伝播を検証したが4週間の観察期間では上位ー下位運動ニューロン間のTDP-43伝播は確認されなかった。
    AAVとCreマウスを用いて、マウス生体内におけるニューロン間、ニューロンーOLG間の伝播を確認することができた。
    in vivo imagingでTDP-43のニューロン間伝播を示唆する現象を捉えることができた。
    CamKII-Creマウスの上位運動ニューロン特異的、またはChAT-Creマウスにおける下位運動ニューロン特異的にTDPmNLS-GFPとTDPmNLSmRRM-GFPを発現させることができた。
    現在確立しているマウス生体内におけるTDP-43のニューロン間伝播とニューロンーOLG間伝播モデルを用いて、その伝播を修飾する因子の検証を行う。具体的にはニューロンの興奮性の制御やエクソソームを標的として検証する。
    in vivo imagingの結果は対側運動野ニューロンにもCre発現が見られるためAAV自体の伝播が否定できないため、光遺伝学的に一側にのみCreを発現させるモデルの構築を行う。
    上位ー下位運動ニューロン間の伝播の検証は観察期間を伸ばし、さらにニューロンの興奮性を高めて検証を進める。

  9. 免疫介在性ニューロパチーの疾患活動性を反映する新規バイオマーカーの開発

    Grant number:20K07864  2020.4 - 2023.3

    科学研究費助成事業  基盤研究(C)

    飯島 正博, 小池 春樹, 勝野 雅央

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    本研究の狙いは免疫介在性ニューロパチーの治療適正化である。CIDP患者の病極期における生検神経から遺伝子発現プロファイルを作成する。また軸索障害を反映するニューロフィラメント-lightによる病態活動性マーカーの有用性を検証する。さらに動物モデルの免疫担当細胞のpopulation解析から病態最上流のpopulationを特定し、これらの免疫学的ネットワークの解明を目指す。
    本研究の狙いは免疫介在性ニューロパチーの治療適正化である。神経領域疾患におけるニューロフィラメント-light(NF-L)は軸索障害を反映するマーカーとして期待されるが、本研究では代表的な免疫介在性ニューロパチーであるCIDPにおいて微量なNF-Lを血清から測定することで、侵襲の少ない病態活動性マーカーとしての有用性を検証する。これまでに58例のCIDP患者の病極期ならびに治療後における血清NF-Lを測定し、CIDPの各亜型と正常コントロール例、臨床スコア、神経伝導検査所見、神経生検の病理所見との関連性を解析した。その結果、NF-Lは正常コントロールより有意な増加を示すとともに、mRSスコアと髄液蛋白と正の相関を認めた。またIgG4自己抗体(抗NF155抗体)陽性例は陰性例より高値を示した。神経伝導検査所見では、NF-Lは脛骨神経の複合筋活動電位振幅と負の相関を示し、神経生検の病理所見からは軸索変性に強い相関を示した。
    また、本研究のもう一つの課題として、CIDPマウスモデルの免疫担当細胞のpo pulation解析から病態最上流のpopulationを特定し、これらの免疫学的ネットワークの解明を目指している。これまでに発症前より病変部坐骨神経においてCD4陽性T細胞とCD68陽性細胞の出現が確認され、これらは相互にmergeする傾向を示した。その後の病極期付近でCD19陽性B細胞が出現するが、FCM解析からこれらはBregに分類されるpopulationが含まれる可能性が示されている。またCD8陽性T細胞はCD4, CD68陽性細胞とは独立して中期より病変部への浸潤が認められた。これより、病態の初期にはマクロファージもしくはAPCとCD4陽性T細胞が病態の主座を担う可能性が示唆され、その後は他のpopulationによる病勢修飾や病態の移行の可能性が示唆された。
    本研究は2つのアプローチにより、CIDPをはじめとする免疫介在性ニューロパチーの病態解明と新規バイオマーカー候補を探索する。1つはCIDP患者血清を用いた超精密ELISA(Simoa system)によりCIDPにおけるNF-Lの変動を鑑別疾患ならびに各亜型間で重症度、神経伝導検査、生検病理所見との相関解析により評価し、疾患活動性・病勢評価としての有用性を検証した。これらはすでに論文(J Neurol. 2021. doi: 10.1007/s00415-021-10537-2., online ahead of print)されたことから、本研究は当初の計画以上に進展している。
    また、CIDPモデルマウスを用いた評価についても、当初計画した各病期における病態を構成する免疫populationの解析は完了し、治療的介入試験として生理食塩水をコントロールとして、完全型免疫グロブリン腹腔内投与時における臨床症状や坐骨神経における病理解析、さらに同部位における浸潤免疫細胞のpopulation解析を行いつつあり、こちらも当初の計画以上の進展を得ている。
    CIDPにおいて多くの患者血清を有することから、今回の対象であるNF-L以外にCIDPの病勢や臨床像を反映するバイオマーカー探索を行う予定である。人ならびにマウスサンプルにCIDP患者血清を反応させた候補バンドを切り出し、質量解析により候補分子を特定する。すでに複数の候補分子が特定されつつあり、なかでも候補分子XについてはELISAならびにウェスタンブロット法による患者血清との反応性も確認しており、CIDPの特定の亜型におけるあらたな診断的バイオマーカーの可能性と病因としての検証を今後試みる予定である。
    CIDPマウスモデルに関しては、完全型グロブリンによる免疫修飾を確認したことから、今後はFcやF(ab')2といった断片化グロブリンを人為的に作製して投与することで、どの断片が免疫修飾能を有するかの検証を試みる予定である。

  10. 超早期ライフステージに着目した神経変性疾患の時空間特異性の解明

    Grant number:20H00527  2020.4 - 2023.3

    科学研究費助成事業  基盤研究(A)

    勝野 雅央, 佐橋 健太郎, 井口 洋平

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    Grant amount:\45630000 ( Direct Cost: \35100000 、 Indirect Cost:\10530000 )

    神経変性疾患に共通する病態的特徴は、特定のニューロンが選択的に変性すること(空間的特異性)および中高年以降に遅発性に発症・進行すること(時間的特異性)である。本研究では、運動ニューロン疾患を研究対象とし、マウスモデルや患者由来iPSCを用い、マルチオミクスやDREADD、脳Caイメージングなどを駆使して超早期ライフステージにおけるニューロン変性分子病態とその年齢依存性変化を解明することで、神経変性における分子病態の真の起点を明らかにする。さらに、ニューロン分化と変性の関連を解析することで、選択的ニューロン死の本質的原因を究明する。
    本研究の目的は、運動ニューロン疾患のマウスモデルや患者由来iPSCにおけるマルチオミクスやDREADD、脳Caイメージングなどを解析することにより、超早期ライフステージにおけるニューロン変性分子病態とその年齢依存性変化を解明することである。本年度は、球脊髄性筋萎縮症(SBMA)マウスモデルにおける超早期病態解析と核酸医薬による治療効果解析とを行った。その結果、SBMAモデルマウスではP1の時点から変異アンドロゲン受容体の核内集積が脊髄運動ニューロンや骨格筋において認められ、P7においてそれらの増強が認められた。超早期病態の解明のため、P1雄マウスの脊髄を用いてRNAseqを行ったところ、後シナプス関連分子やCaチャンネルなどの発現増加が認められ、SBMA患者iPSC由来運動ニューロンでみられた変化と合致していた。今後、SBMAの超早期におけるシナプス病態について解析を進める予定である。
    一方、P1の雄マウスにアンドロゲン受容体に対するアンチセンス核酸(ASO)を脳室内投与したところ、P7において脊髄のアンドロゲン受容体集積が抑制されたが、骨格筋ではアンドロゲン受容体蛋白質の減少は明らかでなかった。この効果はP21にはすべて消失していたが、経時的運動機能解析においては持続的な運動機能の改善が認められ、生存期間も延長した。逆に、P1の雄マウスにテストステロンを皮下投与したところ、運動機能の増悪と生存期間の短縮が認められた。P7においてRNAseqを行うと、ASO治療を行ったマウスのmRNA発現パターンは野生型マウスに近づいていることから、遺伝子発現のレベルでも効果が確認されたと考えられる。これらの結果は、超早期の病因蛋白質の発現量がその後長期にわたる神経筋変性に影響を及ぼすことを示していると考えられる。
    P1マウスに対するASO治療効果が予想以上に明確に検出でき、RNAseq解析も進んでいる。また、患者iPSC由来ニューロンのデータとも類似したデータが得られ、シナプスに焦点を当てた解析を進めることができている。
    今後はSBMAマウスP1~P7におけるシナプス異常について、免疫染色などを用いて検証する予定である。さらに、RNAseqのクラスター解析で抽出された遺伝子群の機能解析を行うことで、新生児期に変異アンドロゲン受容体がどのような経路を介して運動ニューロン変性を惹起するのかを明らかにしていく。

  11. 非全身性血管炎性ニューロパチーの病態解明と新規治療法の開発

    Grant number:20K07882  2020.4 - 2023.3

    科学研究費助成事業  基盤研究(C)

    小池 春樹, 勝野 雅央, 飯島 正博

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    本研究ではNSVNの臨床病理像を明らかにするとともに、疾患マーカーとなる抗体の探索や治療反応性の検討などを行う。はじめに、患者レジストリを構築してNSVNの臨床病理像の検討を行う。抗体の探索ではマウスの神経組織と患者血清で特異的に反応する蛋白質の同定を行い、自己抗体が認識する抗原を同定した後、自己抗原の組織内分布を解析する。抗原候補蛋白をマウスに投与することによってNSVNモデルマウスを作成し、病態解析を行う。さらに、NSVNにおけるエクリズマブのにおける有効性と安全性についても検討する。
    血管炎は様々な原因で発症し、神経障害、特に末梢神経障害(ニューロパチー)を高頻度にきたすことが知られている。急性または亜急性に進行する場合が多く、治療により原疾患を寛解に導いても神経障害が残存することも多いことから、早期からの治療介入が必要な疾患である。末梢神経系のみが障害される非全身性血管炎性ニューロパチー(non-systemic vasculitic neuropathy; NSVN)は1987年にDyckらによって初めて報告された概念であり、顕微鏡的多発血管炎と並んで血管炎によるニューロパチーの主要な原因と考えられるようになっている。しかし、NSVNは非特異的なニューロパチーの病像を呈し、血液検査でもCRP値や血沈も含めて異常所見に乏しいことから診断に難渋する場合が多い。さらに治療反応性を示すマーカーもないことから、治療方針の確立も困難である。このことから、NSVNの早期診断、早期治療につなげることのできるバイオマーカーを明らかにする必要がある。
    本研究ではNSVNの臨床病理像を、日常診療の場で鑑別が問題となる顕微鏡的多発血管炎と比較することにより全身性血管炎との連続性を明らかにするとともに、疾患マーカーとなる抗体の探索や前向きの治療反応性の検討などを行う。はじめに、患者レジストリを構築してNSVNの長期予後も含めた臨床像・病理像の検討を行う。特に、顕微鏡的多発血管炎による末梢神経障害と比較することによって全身性血管炎との連続性を明らかにする。抗体の探索ではマウスの神経組織と患者血清で特異的に反応する蛋白質の同定を行い、自己抗体が認識する抗原エピトープを同定した後、自己抗原の組織内分布を解析する。抗原候補蛋白をマウスに投与することによってNSVNモデルマウスを作成し、病態解析を行う。さらに、NSVNにおけるエクリズマブのにおける有効性と安全性についても検討する。
    名古屋大学神経内科にコンサルテーションのあった症例も含めて発症年齢、初発症状、進行期間、障害の進展様式、重症度などの臨床像、および治療反応性も含めた長期予後を検討し、顕微鏡的多発血管炎に伴うニューロパチー、特にANCA陰性例と比較した。NSVNに関しては今後も新規の症例のconsultationが年間10例程度はあると予想され、最終的には50例程度での長期予後の検討を行う方針である。具体的には当院で診断したNSVN症例の既存例も含めた登録システムを構築して、臨床症候・電気生理学的検査所見・血液検査所見などの前向きの情報収集と生体試料のサンプリングを定期的に行う。さらに、腓腹神経生検の検体を用いて有髄線維密度と無髄線維密度の測定を含めた形態学的検討を行うとともに、ホルマリン固定および凍結の検体を用いて免疫組織化学的検討を行った。具体的には、顕微鏡的多発血管炎の初期病変といわれているANCA陽性好中球の小血管内皮への接着に伴う内皮細胞の障害の有無を光顕レベルでの検討、および電顕を用いた超微形態学的な検討にて確認した。NSVNと顕微鏡的多発血管炎では両者ともに壊死性血管炎の所見が見られるものの、血管内皮に接着した好中球はNSVNではほとんど認めないのに対し、顕微鏡的多発血管炎では多く見られ、後者では抗好中球細胞質抗体が病態に重要な役割を果たしていることが推測された。一方、補体の沈着をC3、C4、C5、MACなどに対する抗体を用いて検討したところ、顕微鏡的多発血管炎と比較してNSVNで補体沈着が多く見られたことからNSVNにおける補体を介した病態の重要性が示唆された。さらに末梢神経系における血管炎は神経上膜の細血管で生じることから、画像解析ソフトを用いて神経上膜の面積を測定し、好中球の接着や補体が沈着血管の数を定量化して顕微鏡的多発血管炎に伴うニューロパチーと比較した。
    NSVNの長期予後も含めた臨床像・病理像の検討に関しては今後も継続していく。症例のリクルートに関しては、名古屋大学大学院医学系研究科には、NSVNのみならず血管炎関連疾患全般に関して多数の生検および剖検例の蓄積がある。また、NSVNの認知度の高まりにつれて、今後も新規の症例が多数加わることが予想され、多数例での臨床病理学的なアプローチのみならず、治療反応性も含めた前向きの臨床研究も可能となる。免疫組織化学的検討や免疫電顕に必要な光学および電子顕微鏡をはじめとする機器は神経内科学教室および分析機器部門に設置されており、今後も本研究のために積極的に活用していく。これらの研究から得られた知見をもとに、NSVNを発症させる標的抗原の確定とモデルマウスの解析、治療への展開を目指す。本研究の研究分担者および研究協力者は研究代表者と同一の施設に在籍し、定期的に連絡調整を行っている。研究分担者の飯島正博は他の研究協力者とともに末梢神経疾患の生検/剖検検体を用いた病態解析とモデルマウスの解析を行っている。モデルマウスの解析手法は研究分担者である勝野雅央らによって確立されており、この手法を活用する。また、研究成果は国内および海外での学会で積極的に発表するとともに、名古屋大学神経内科ホームページ、機会があれば報道機関などを通して研究成果を社会・国民に発信していく。
    以上のような計画遂行で、NSVNの病態を究明し新規治療法の開発へつなげることができると考える。

  12. 候補遺伝子アプローチによる筋萎縮性側索硬化症病態関連遺伝子の探索

    Grant number:19K07973  2019.4 - 2022.3

    科学研究費助成事業  基盤研究(C)

    中村 亮一, 藤内 玄規, 勝野 雅央, 熱田 直樹

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    筋萎縮性側索硬化症(ALS)は代表的な神経難病である。近年、家族性ALS原因遺伝子が数多く同定され、ALSの病態解明と根治的治療法開発の重要な手がかりになりつつある。しかし、大部分を占める孤発性ALSの関連遺伝子は十分に解明されていない。パーキンソン病とゴーシェ病との関連のように、異なる疾患との関連が遺伝子解析により示される場合がある。本研究では、多数のALS患者のDNA検体を用いて、ALS以外の神経変性疾患、筋疾患、末梢神経疾患の関連遺伝子の網羅的解析を行い、それらの変異とALSとの関連を明らかにする。新たなALS関連遺伝子の同定により病態解明、治療法開発の重要な手がかりを提供できる。
    筋萎縮性側索硬化症(ALS)は代表的な神経難病であるが、近年病態関連遺伝子が数多く同定されている。30種類以上判明しているALS関連遺伝子はRNA代謝やタンパク質品質管理などの機能に分類でき、依然謎であるALSの病態解明と根治的治療法開発の重要な手がかりになりつつある。しかし判明したALS関連遺伝子の多くは家族性ALSの解析から同定されており、大部分を占める孤発性ALSの関連遺伝子は十分に分かっていない。より多くのALS関連遺伝子を探索同定する必要があるが、通常そのためには数千例以上の規模でのゲノムワイド関連解析が必要であり、容易ではない。しかしパーキンソン病とゴーシェ病との関連のように、異なる疾患の関連が遺伝子により示される場合があり、その発想を用いた候補遺伝子アプローチにより、効率よくALS関連遺伝子を探索同定できる可能性がある。
    本研究では、多数のALS患者のDNA検体及び縦断的臨床像を用いて、ALS以外の神経変性疾患、筋疾患、末梢神経疾患の関連遺伝子の網羅的解析を行い、それらの変異とALSとの関連を明らかにする。
    令和2年度においては、孤発性ALS807例の網羅的遺伝子解析データを用いて、遺伝性ニューロパチーと遺伝性痙性対麻痺の原因遺伝子であるKIF5A遺伝子に関して解析を行い1例(0.12%)に新規スプライスサイト変異(c.2993-3C>A)を同定し、病的変異であることを確認し、論文報告した。また、ALS患者の遺伝子解析データから3例に、筋疾患や末梢神経疾患の原因遺伝子でもあるVCP遺伝子変異を認め、うち1例は新規遺伝子変異(p.Asp98Val)であり、まとめて論文報告した。また、脂質代謝に関連する遺伝子であるACSL5を新規ALS疾患関連遺伝子として同定し、脊髄小脳変性症3型(SCA3)の原因遺伝子であるATXN3もALSの疾患関連遺伝子として同定した。
    既に当初の想定数以上の孤発性ALS患者に対して網羅的な遺伝子解析を施行している。
    抽出されたvariantを今日までにALSやFTLD、認知症、遺伝性痙性対麻痺、末梢神経障害、筋疾患の発症の原因として同定されているもの、ALSや上記疾患の発症には無関係であると同定されているもの、またそのどちらでもないものに分類し、それぞれサンガー法で変異の有無を確認している。いくつか疾患関連遺伝子を見いだし、論文報告している。他にもいくつか疾患関連遺伝子の候補を同定しており、それらの機能解析も進めている。
    令和3年度も引き続き、遺伝子解析未施行の残りの孤発性ALS検体に対して網羅的なシーケンスを行った上で抽出されたvariantについての解析を施行する。また、現在見いだしている疾患関連遺伝子の候補に関して分子生物学的検討を行ったり、variantを持つ症例と臨床像との関連も検討する。遺伝的背景が明らかになった患者のうち剖検組織のある患者がいれば、病理像も検討する予定である。

  13. Understanding of defective developmental gene regulation responsible for motor neuron degeneration

    Grant number:19H03545  2019.4 - 2022.3

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  14. 異常タンパク分解系を標的とした運動ニューロン疾患治療薬の開発

    Grant number:19K06523  2019.4 - 2022.3

    科学研究費助成事業  基盤研究(C)

    藤内 玄規, 勝野 雅央, 佐橋 健太郎

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    球脊髄性筋萎縮症(SBMA)は致死的運動ニューロン疾患であり、変異アンドロゲン受容体(AR)タンパク質の神経細胞核内への蓄積が病因と考えられる。この変異タンパク質の蓄積にはタンパク質分解機構が深く関わっているが蓄積過程への作用は不明な点も多い。そこで本研究では(1)標識した変異ARを持つモデル細胞を用いて変異タンパク質の発現と核内蓄積、凝集体形成過程及びタンパク質分解機構に関わる分子の病態への関与の解析、(2)モデル線虫を用いた核内蓄積、凝集体形成過程と運動機能への影響の解析を行い、SBMAにおける変異タンパク質がもたらす神経細胞の機能異常・細胞死のメカニズムを可視化し、治療法の開発を目指す。
    球脊髄性筋萎縮症(SBMA)などの運動ニューロン疾患は根本的治療法のない致死的神経変性疾患である。これらの疾患の特徴として神経細胞の変性と蓄積が見られる。SBMAにおいては変異アンドロゲン受容体(AR)タンパク質の神経細胞核内への蓄積が病因と考えられる。
    本研究では変異ARタンパク質の蓄積過程を可視化することで、SBMAの病態解明とタンパク質品質管理機構に関わる分子を標的とした効果的で臨床応用可能な治療法の開発を目指す。さらに様々な長さのCAGリピートを持つAR遺伝子を導入したSBMA細胞モデルを作成し、ARタンパク質のポリグルタミン鎖の長さの違いによるタンパク質品質管理機構関連分子との相互作用の変化を解明する。
    本年度はCAGリピートが97に異常伸長したARタンパク質(AR-97Q)の核内移行を可視化したSBMA培養細胞モデルを用い、異常タンパク質の核内移行と複数のタンパク質分解系に注目しドラッグスクリーニングすることで従来の作用機序とは異なる治療候補化合物や新たな分解系を活性化する治療候補化合物を見出した。またCAGリピートが伸長すると特定のタンパク質品質管理機構に関わる分子が変異AR凝集体に含まれていることを確認し、その量がリピートの長さに依存せず、特定のリピートで多くなることも判明した。
    今後SBMAモデル動物としてAR-97QまたはAR-24Qを導入したSBMAモデル線虫を作成し、すでに樹立しているSBMAマウスとともに候補化合物の治療効果を検討する。
    本年度はCAGリピートが異常伸長したARタンパク質の核内移行を可視化したSBMA培養細胞モデルを用い、タンパク質品質管理機構に関わる重要な分子との作用を検討した。異常タンパク質の核内移行と複数のタンパク質分解系をモニタリングしドラッグスクリーニングすることで従来の作用機序とは異なる治療候補化合物や新たな分解系を活性化する治療候補化合物を見出した。
    前年度にCAGリピートが伸長すると特定のタンパク質品質管理機構に関わる分子が変異AR凝集体に含まれていることを確認したが、本年度の解析ではその分子の含まれる量がリピートの長さに依存せず、特定のリピートで多くなることも判明した。
    また我々が注目しているタンパク質品質管理機構に関わる分子の遺伝子変異が他の神経変性疾患に関連しているとの報告があったためSBMAと同じ運動ニューロン疾患であるALSの患者をエクソーム解析したところ複数の新規の遺伝子変異を見出した。現在SBMAにおける遺伝子変異の影響を解析中である。
    本年度も予定どおり細胞モデルにおいて候補化合物を見出しており本研究はおおむね順調に進展している。
    本年度までの解析で見出したタンパク質品質管理機構を強化する可能性のある候補化合物を当研究室で作成された異常伸長したCAGリピートを持つヒト全長AR遺伝子を発現するSBMAモデルマウスに投与し検証する。このマウスの表現型はRotarod法、Cage activity法、握力測定、体重変化、生存率の5つのパラメーターで測定するとともに組織における変異AR、タンパク質品質管理機構に関わる分子の発現量をウェスタンブロット、Real-time PCR、免疫組織化学を用いて解析し、候補化合物の治療効果を解析する。また現在作成中のSBMAモデル線虫を樹立し、SBMAモデルマウスとともに候補化合物の治療効果を解析する予定である。
    変異ARのリピート長と毒性の関連解析は、本年度実施したAR遺伝子のCAGリピートの伸長と変異AR凝集体に含まれる分子の解析をさらに進め、リピート長による毒性の変化の解明を目指す。
    また本年度実施したALS患者のエクソーム解析で見出したタンパク質品質管理機構に関わる分子の遺伝子変異をSBMAにおいても解析し、運動ニューロン病共通の病態形成メカニズムを探索する。

  15. Elucidation of the progression of multiple system atrophy by head MRI

    Grant number:19K07960  2019.4 - 2022.3

    Grants-in-Aid for Scientific Research  Grant-in-Aid for Scientific Research (C)

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  16. Understanding of intrinsic readthrough mechanisms of nonsense mutations for its therapeutic applications

    Grant number:18K19512  2018.6 - 2020.3

    Grant-in-Aid for Challenging Research (Exploratory)

    Sahashi Kentaro

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    We identified a late-onset congenital myopathy due to a novel homozygous nonsense mutation in SEPN1. A high incidence of this autosomal recessive disorder observed in 3 cases out of 5 siblings was attributed to copy-neutral LOH in chromosome 1p36.11. Despite the loss-of-function mutation, SEPN1 was highly expressed in patients' skeletal muscle. The recoding of the mutation at the RNA level was detected in both muscle and blood cells, and the RNA editing enzyme ADAR2 was shown to catalyze this nucleotide conversion in patient fibroblasts. Furthermore, we uncovered high expression of SEPN1 mRNA and the possible recoding event in muscle of a model mouse. The finding of ADAR2-mediated attenuation of the pathology would further provide insights into alternative therapeutic approaches for genetic disorders.

  17. Long-term effect of leuprorelin acetate on the patients with spinal and bulbar muscular atrophy

    Grant number:18K07523  2018.4 - 2021.3

    Grants-in-Aid for Scientific Research  Grant-in-Aid for Scientific Research (C)

    Hashizume Atsushi

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    Spinal and bulbar muscular atrophy (SBMA) is one of rare neuromuscular diseases. In 2017, leuprorelin acetate which has disease-modifying effect on SBMA was approved in Japan, however, there is not enough information to demonstrate its efficacy or appropriate usage of this drug. The purpose of the study is to develop a real-world database to accumulate more information to demonstrate its efficacy.The principal outcome measurement to evaluate disease severity was ALSFRS-R.A total of 392 SBMA patients were enrolled. Although the results of a slope analysis for total population presented that there was no significant difference between pre- and post-treatment of leuprorelin acetate, the analysis for subpopulation who have enough observational records (more than two years before and after administration) revealed that the drug might slow disease progression (disease progression slope before intervention; -0.528 ± 0.634/ year: slope after intervention; -0.292 ± 1.471/ year (p = 0.3185)).

  18. 球脊髄性筋萎縮症に対する骨格筋トランスポーターを標的とした治療法開発

    Grant number:18K07497  2018.4 - 2020.3

    土方 靖浩

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    本研究の目的は、球脊髄性筋萎縮症(SBMA)患者の、骨格筋内クレアチン取り込みを促進することによる病態改善をめざした新規治療法開発である。平成30年度では、クレアチントランスポーターSLC6A8を標的とした化合物スクリーニング(名古屋大学創薬科学研究所および第一三共株式会社TaNeDSプログラム)を行い、SLC6A8の転写活性および蛋白質発現を上昇させる化合物を同定した。
    平成31年度(令和1年度)では、SBMA患者のfibroblastを用いたviability改善効果や過去の薬理試験結果等から、最終的に3化合物(T8, T38, T40)について、SBMAマウスモデル(AR-97Q)を用いて検討を行った。薬理試験の結果を参考にして各化合物について2段階の投与量を設定し、週5回投与を行った上で骨格筋SLC6A8のmRNA量を測定したところ、1mg/kg/dayのT38を投与した際にmRNA量の上昇をみとめたため、T38(1mg/kg/day)の長期投与を行った。T38のゾンデによる強制経口投与を6週齢から一日一回、週に5日間行い、体重、握力、ロタロッド試験による運動機能評価を行った。T38投与による運動機能改善の程度は限定的であり、投与量を増量して評価を実施した。T38は血糖低下作用を有するため、2mg/kg/day と5mg/kg/dayの各群で空腹時血糖と薬剤投与2時間後の血糖を測定した。5mg/kg/dayの投与群では半数のマウスで低血糖を認めたため運動解析を中止した。2mg/kg/day投与にて運動機能評価を実施した結果したところ、1mg/kg/dayの投与群と同様に効果は限定的であった。T38によるSBMAマウスモデルの神経変性抑制効果は限定的であり、かつ低血糖の副作用を有するため高用量を投与することも難しいことから臨床応用は困難であると判断した。

  19. Deciphering the mechanism of neuropathy and development of novel therapies in familial amyloid polyneuropathy

    Grant number:17K09777  2017.4 - 2020.3

    Koike Haruki

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    Transthyretin (TTR) Val30Met familial amyloid polyneuropathy (FAP) (FAP ATTR Val30Met) is the most common type of FAP. Long amyloid fibers were abundant in the conventional early-onset Val30Met cases from endemic foci, whereas the amyloid fibrils were generally short in the late-onset Val30Met and non-Val30Met cases from non-endemic areas. The distortion of Schwann cells close to amyloid fibril masses was conspicuous, particularly in cases with long amyloid fibrils. Findings suggestive of the disruption of blood-nerve barriers, such as the loss of tight junctions and the fenestration of endothelial cells, were frequently found particularly in late-onset FAP cases. These findings suggest that direct insult of amyloid fibrils causes Schwann cell damage resulting in the predominant loss of small-fiber axons characteristic of early onset cases. In addition, vasculopathy may also participate in the pathogenesis of neuropathy, particularly in late onset cases.

  20. Discovery of novel target and disease modifying therapy for ALS/FTLD using neuro-specific transcriptome analysis

    Grant number:17K09753  2017.4 - 2020.3

    IGUCHI YOHEI

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    ALS and FTLD are progressive neurodegenerative diseases and the causes are unknown. The genetic and neuropathological studies suggest that loss of functions of TDP-43 or TBK1 are related to the neurodegeneration. We established a novel transgenic mouse, in which GFP-tagged ribosomal protein (RPL10a) and Cre are expressed under synapsin promotor. This mouse allows us to perform neuron-specific polysome (mRNA-ribosome complex) in the conditional TDP-43/TBK1 knockout mice. We validated the expression profile of GFP-RPL10a of the transgenic mouse and analyzed the neuron-specific transcriptome data of the conditional knockout mice.

  21. Elucidation of defective neural communication in motor neuron diseases

    Grant number:17H04195  2017.4 - 2020.3

    Katsuno Masahisa

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    Grant amount:\16770000 ( Direct Cost: \12900000 、 Indirect Cost:\3870000 )

    Nuclear depletion of TDP-43 is the histopathological hallmark of amyotrophic lateral sclerosis. In this research, we showed that loss of TDP-43 inhibited exocytosis by down-regulating CaV1.2 calcium channels, thereby reducing early-phase insulin secretion. Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by a CAG repeat expansion in the androgen receptor (AR) gene. In this research, we showed that DNA methyltransferase 1 is highly expressed in the motor neurons of an SBMA mouse model, and in patients with SBMA. Treatment with RG108, a DNA methylation inhibitor, ameliorated the viability of SBMA model cells and s the motor function of SBMA mice. We also found that the level of phosphorylated Src was markedly increased in the spinal cords and skeletal muscles of SBMA mice prior to the onset, by utilizing a phosphoprotein assay. Intraperitoneal administration of a Src kinase inhibitor improved the behavioral and histopathological phenotypes of SBMA mice.

  22. Therapeutic development based on factors affecting progression and prognosis of amyotrophic lateral sclerosis

    Grant number:17K09778  2017.4 - 2020.3

    Atsuta Naoki

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    We identified clinical factors affecting progression and prognosis of ALS patients such as disease type and decline rate of the severity scale (ALSFRS-R). We also identified ADMA/L-arginine ratio in cerebrospinal fluid as a biomarker for predicting prognosis of ALS patients. The gene polymorphisms related to the prognosis of ALS patients were verified, and the novel ALS-related genes were verified in Japanese population. These contribute to therapeutic intervention clues and clinical trial design. In addition, the effects of therapeutic intervention (tracheostomy invasive ventilation therapy), which is difficult to verify in randomized controlled trials, on the prognosis of ALS patients were examined using accumulated longitudinal clinical data.

  23. Analysis of genes affecting cognitive function in sporadic ALS

    Grant number:17K16116  2017.4 - 2019.3

    Nakamura Ryoichi

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    Recent studies have shown that about 50% of patients with amyotrophic lateral sclerosis (ALS) have some cognitive impairment, and approximately 10-15% of ALS patients meet the clinical criteria of frontotemporal dementia (FTD) and are diagnosed as ALS-FTD. We performed genetic analysis on 713 ALS patients using a next-generation sequencer. Among the 713 ALS patients, 9 cases (1.26%) harbored a mutation of the TBK1 gene, and some cases with the TBK1 mutation were diagnosed as ALS-FTD. Moreover, other 3 cases harbored a mutation of the VCP gene. Of the 3 cases, 2 cases had inclusion body myopathy or FTD patients in their families. The remaining one had behavioral disturbance and was diagnosed as ALS-FTD. These results suggested that the mutations of the TBK1 and VCP genes are important in the genetic background of ALS with cognitive dysfunction.

  24. Development of biomarkers of sleep disturbance in Parkinson's disease using salivary melatonin

    Grant number:16K09713  2016.4 - 2019.3

    NAKAMURA TOMOHIKO

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    We measured salivary melatonin and examined its feasibility as a biomarker of sleep disturbance and cognitive dysfunction in Parkinson's disease (PD).
    Patients with PD often have abnormal melatonin secretion such as higher melatonin levels during the day than those in the morning or night or a forward shift of the phase of melatonin secretion before sleeping. Melatonin levels may be a biomarker of sleep disturbance in PD. In addition, elevated melatonin levels during the day are associated with cognitive dysfunction. Thus, circadian rhythm disorder may be associated with cognitive impairment in PD. Furthermore, melatonin levels before sleeping are positively correlated with disease duration and Hoehn-Yahr severity, and elevated melatonin levels may represent the spread of brain lesions in PD.

  25. Elucidation of the axonal degenerative mechanism focusing on the paranodal molecules in immune-mediated neuropathy

    Grant number:16K09693  2016.4 - 2019.3

    Iijima Masahiro

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    We have conducted a consortium called “CIDP-J” at 16 domestic universities for research of CIDP. More than 200 cases retrospectively and prospectively collected were registered during the research period. Clinical information such as age at onset, sex, progression type, INCAT score, R-ODS, grasp power, MRC score, responsiveness to each therapeutics have been collected. The breakdown of IgG4-subclass autoantibodies (NF155, CNTN1) that target paranode is newly clarified in this study, and it can be useful for future criteria and guideline as well as severity classification.
    About NOD B7-2 knockout mice, which are expected as an inflammatory demyelinating model for CIDP, are characterized by progressive posterior weakness with severe infiltration of macrophages as well as lymphocytes in PNS. We analyzed the natural history and fluctuation of the infiltration over time, it leads to the elucidation of pathogenesis reflecting disease activity and irreversible axonal degeneration.

  26. Biomarker study in the early phase patients with spinal and bulbar muscular atrophy

    Grant number:15K09311  2015.4 - 2018.3

    Hashizume Atsushi

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    To develop new disease-modifying drugs for neurodegenerative diseases, a valid, reliable, and sensitive outcome measurement would be necessary. The present study aimed to develop a new composite measurement which reflects disease severty well. In this study, a total of 81 patients with SBMA and 23 normal contols were recruited. In the cross-sectional study, five components, tongue pressure, grip power, %PEF, 4.6 m timed walking test, and %VC, were appropriate to be combined with to develop a new composite mesurement.
    We followed-up the subjects for at most 96 weeks to confirm the usefulness of this new measurement. The results of this longitudinal study indicated that the composite measurement was meaningful as for sensitiveness.

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