Faculty Profiles - MATSUSHITA, Tadashi

Papers - MATSUSHITA, Tadashi

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Use of cyclophosphamide and total lymphoid irradiation combined with cyclosporine in bone marrow transplantation for transfused severe aplastic anemia. Reviewed

"K. Miyamura, S. Kojima, K. Takeyama, T. Matsushita, M. Fukuda, K. Horibe, S. Minami, Y. Morishima, K. Matsuyama and Y. Kodera"

Bone Marrow Transplantation Vol. 3 page： 457-461 1988

Comparison between bone marrow transplantation and immuno-suppressive therapy in treatment of patients younger than 20 years with severe aplastic anemia Reviewed

"Kojima, S., Fukuda, M., Horibe, K., Matsuyama, K., Takeyama, K., Matsushita, T., Miyamura, K., Kimura, Y., Minami, S. and Kodera, Y."

Acta. Haematol. Jpn. Vol. 51 ( 1 ) page： 28-35 1988

Allogeneic bone marrow transplantation for Burkitt's lymphoma (stage IV). Reviewed

"Miyamura, K., Takeyama, K., Matsushita, T., Kimura, M., Minami, S., Yoshida, J., Matsuyama, K., Kodera, Y., Yamauchi, T. and Hiraiwa, A., et al."

"Jpn. J. Clin. Hematol. 29, 149-55, 1988" Vol. 29 ( 2 ) page： 149-55 1988

Cyclosporine as prophylaxis for graft versus host disease in adults undergoing allogeneic bone marrow transplantation Reviewed

"Matsushita, T., Akatsuka, Y., Towatari, M., Takeyama, K., Miyamura, K., Sugihara, T., Minami, S., & Kodera, Y."

Jpn. J. Clin. Hematol. Vol. 30 ( 4 ) page： 429-436 1989

Effects of ceftriaxone on severe infectious complications in hematological disorders. Tohkai Research Group on Infections in Hematological Disorders Reviewed

"Mitomo, Y., Nitta, M., Kodama, H., Kobayashi, M., Ikeda, Y., Kodera, Y., Matsushita, T., Ohara, K., Kamiya, O. and Shirakawa, S., et al."

Jpn J Antibiot Vol. 42 ( 4 ) page： 938-47 1989

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

"Ceftriaxone (CTRX), a new long acting antibiotic in the 3rd generation cephem group, was administered intravenously once or twice a day in daily doses of 1-6 g for at least 3 days to 86 patients with severe infections complicating hematopoietic disorders. Underlying diseases were acute leukemia in 41 cases, chronic leukemia in 3 cases, malignant lymphoma in 19 cases, myeloma in 7 cases and others. Most patients (55 cases) suffered from sepsis or suspected sepsis. As for efficacy rates classified by underlying diseases, the treatment was effective in 61.0% of patients with acute leukemia. As for efficacy rates classified by infections, the treatment was effective in 60.0% of patients with sepsis. No side effects were noted except rash in 2 patients. Abnormal hepatic functions were recognized in 3 patients but were not attributed to the agent in any case. The results indicate that CTRX is a safe and useful antibiotic for the treatment of severe infections accompanied by hematopoietic disorders."

Prolonged thrombocytopenia after autologous bone marrow transplantation Reviewed

"Kojima, S., Fukuda, M., Horibe, K., Matsuyama, T., Takeyama, K., Matsushita, T., Miyamura, K., Sugihara, T., Minami, S., & Kodera, Y."

Jpn. J. Clin. Hematol. Vol. 30 ( 2 ) page： 175-80 1989

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

"Thirty two patients with hematologic malignancies and solid tumors were treated with intensive therapy and autologous bone marrow transplantation. In nine out of 32 patients, it took more than 50 days to achieve a sustained platelet count of 50,000/microliter or greater. Significant associations with poor platelet recovery were found for patient age, diseases, period of cryopreservation, the kinds of eradicative therapy and in vitro purging. But most of these factors overlapped each other in the same patients. No correlation was found between platelet recovery and number of cells or CFU-GM infused."

Severe aplastic anemia remarkably improved by a treatment with antilymphocyte globulin, high-dose methylprednisolone and danazol Reviewed

Miyamura, K., Kojima, S., Takeyama, K., Matsushita, T., Minami, S., & Kodera, Y.

Jpn. J. Clin. Hematol. Vol. 30 ( 1 ) page： 72-77 1989

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

Sixteen-years-old female with severe aplastic anemia received a therapy combined with antilymphocyte globulin (ALG), high-dose methylprednisolone (m-PSL) and danazol. At the hospitalization, hematological examination demonstrated as follows; reticulocyte 21,000/microliters, granulocyte 350/microliters, platelet 10,000/microliters and hypocellular bone marrow. Treatment schedule were 1) m-PSL 1,000 mg (day 1-4), 500 mg (5-8)--then tapered. 2) ALG lg/day (day 4-8) 3) danazol 600 mg/day. During ALG administration, leukocytopenia and thrombocytopenia appeared but thereafter hematological recovery was obtained and the patient was free from supportive care. She developed mild diabetes mellitus and moderate liver dysfunction, nevertheless, both of which were controlled. At 3 months after the beginning of the treatment, hematological examination demonstrated as follows; reticulocyte 236,000/microliters, granulocyte 1,900/microliters, platelet 56,000/microliters and normocellular bone marrow. Although this immunosuppressive therapy was remarkably effective to this patient, immunological relation to the onset of aplastic anemia was not demonstrated in in vitro examination. This combined therapy seems to be effective one for patients with severe aplastic anemia.

The analysis of mutant factor IX gene responsible for the development of inhibitor in hemophilia B. Reviewed

"Matsushita, T."

Hematology & Oncology Vol. 21 ( 4 ) page： 472-477 1990

Gene diagnosis of hereditary bloodcoagulation factor disorders Reviewed

"Matsushita, T., Sugiura, I."

Jpn. J. Clin. Pathol. Vol. 85 page： 114-122 1990

Molecular diagnosis of inherited coagulation disorders--sequence analysis of hemophilia B patients with anti-factor IX antibodies Reviewed

"Tanimoto, M., Matsushita, T., Takamatsu, J. and Saito, H."

"J. Clin. Pathol. 38, 1041-1046, 1990" Vol. 38 ( 9 ) page： 1041-1046 1990

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

Seven hemophilia B patients with anti-factor IX antibodies are studied with molecular means. A total factor IX gene deletion was detected in four patients from three distinct families. Three other patients without detectable gene arrangements were investigated at the DNA sequence level. Enzymatic amplification of the factor IX gene and subsequent DNA sequencing revealed four novel nucleotide mutations in these patients. Patient HB 5 had two point mutations in his factor IX gene. One is located at nucleotide -793 from the translation start (G-A) and the other (C-T) was found in the codon for 191-Gln changing to a termination codon. Patient HB6 had a point mutation (G-A) in the splicing junction of intron g. A2 bp nucleotide deletion was detected in the third exon of the patient HB7 yielding 8 frameshifted amino acids and a stop codon. These results suggested that not only a large deletion of the factor IX gene but also point mutations or small deletion of the gene which may cause the substantial loss of the coding information for the mature protein are involved in the development of anti-factor IX antibodies in hemophilia B patients.

DNA sequence analysis of three inhibitor-positive hemophilia B patients without gross gene deletion. Identification of four novel mutations in factor IX gene. Reviewed

"Matsushita, T., Tanimoto, M., Yamamoto, K., Sugiura, I., Hamaguchi, M., Takamatsu, J., Kamiya, T. and Saito, H."

J Lab Clin Med. Vol. 116 page： 492-497 1990

Cytomegalovirus-induced gastritis in a bone marrow transplant patient. Reviewed

"Minami, H., Matsushita, T., Sugihara, T., Kodera, Y., Sakai, S. and Shimokata, K."

INTERNAL MEDICINE Vol. 29 page： 433-435 1990

Molecular genetics of hemophiliacs Reviewed

"Matsushita, T. and Saito, H."

Hematology & Oncology Vol. 22 ( 1 ) page： 129-140 1991

Homozygous protein C deficiency: identification of a novel missense mutation that causes impaired secretion of the mutant protein C. Reviewed

"Yamamoto, K., Matsushita, T., Sugiura, I., Takamatsu, J., Iwasaki, E., Wada, H., Deguchi, K., Shirakawa, S. and Saito, H."

J. Lab. Clin. Med. Vol. 119 page： 682-689 1991

"Three distinct point mutations in the factor IX gene of three CRM+ hemophilia B patients (Factor IX BMNagoya 2, B+ Nagoya 3 and 4)." Reviewed

"Hamaguchi, M., Tanimoto, M., Matsushita, T., Yamamoto, K., Sugiura, I., Takamatsu, J., Ogata, T., Kamiya, T. and H., S."

Thromb. Haemost. Vol. 65 page： 514-520 1991

Genotype establishments for protein C deficiency by use of a DNA polymorphism in the gene. Reviewed

"Yamamoto, K., Tanimoto, M., Matsushita, T., Kagami, K., Sugiura, I., Hamaguchi, M., Takamatsu, J. and Saito, H."

Blood Vol. 77 page： 2633-2636 1991

Direct carrier detection in hemophilia B kindreds: Use of modified primers (mutagenic primers) for enzymatic amplification of the factor IX gene. Reviewed

"Matsushita, T., Tanimoto, M., Yamamoto, K., Sugiura, I., Takamatsu, J., Kamiya, T. and Saito, H."

Thromb. Res. Vol. 63 page： 355-361 1991

A female hemophilia A combined with hereditary coagulation factor XII deficiency: a case report. Reviewed

"Matsushita, T., Takamatsu, J., Kagami, K., Takahashi, I., Sugiura, I., Hamaguchi, M., Kamiya, T. and Saito, H."

Am. J. Hematol Vol. 39 page： 137-141 1991

Mammalian expression systems of human blood coagulation factors. Reviewed

"Matsushita, T. and Emi, N."

Jpn. J. Thromb. Hemost. page： 546-549 1991

Elevation of factor VII activity and mass in coronary artery disease of varying severity Reviewed

"Suzuki, T., Yamauchi, K., Matsushita, T., Furumachi, T., Furui, H., Tsuzuki, J. and Saito, H."

Clin. Cardiol. Vol. 14 page： 731-736 1991

Three distinct candidate point mutatations of the von Willebrand factor gene in four patients with type IIA von Willebrand disease Reviewed

"Sugiura, I., Matsushita, T., Tanimoto, M., Takahashi, I., Yamazaki, T., Yamamoto, K., Takamatsu, J., Kamiya, T. and Saito, H."

Thromb. Haemost. Vol. 67 ( 6 ) page： 612-617 1992

Impaired secretion of the elongated mutant of protein C (protein C-Nagoya). Molecular and celluler basis for hereditary protein C deficiency. Reviewed

"Yamamoto, K., Tanimoto, M., Emi, N., Matsushita, T., Takamatsu, J. and Saito, H."

J. Clin. Invest. Vol. 90 page： 2439-2446 1992

Construction and its expression of a new retroviral vector containing a human blood coagulation factor IX cDNA Reviewed

"Matsushita, T., Emi, N., Takahashi, I., Takamatsu, J. and Saito, H."

Thromb. Res. Vol. 69 page： 387-393 1993

A phenotypically neutral dimorphism of protein S: the substitution of Lys155 by Glu in the second EGF domain predicted by an A to G base exchange in the gene Reviewed

"Yamazaki, T., Sugiura, I., Matsushita, T., Kojima, T., Kagami, K., Takamtsu, J. and Saito, H."

Thromb. Res Vol. 70 page： 395-403 1993

*Impaired human tissue factor-mediated activity in blood clotting factor VIINagoya (Arg304 -> Trp). Evidence that a region in the catalytic domain of factor VII is important for the association with tissue factor) Reviewed

"Matsushita, T., Kojima, T., Emi, N., Takahashi, I. and Saito, H."

J. Biol. Chem. Vol. 269 ( 10 ) page： 7355-7363 1994

von Willebrand's factor and von Willebrand's diesease Reviewed

"Matsushita, T., Dong, Z. and Sadler, J. E."

Current Opinion in Hematology Vol. 1 page： 362-368 1994

*Identification of amino acid residues essential for von Willebrand factor binding to platelet glycoprotein Ib. Charged-to-alanine scanning mutagenesis of the A1 domain of human von willebrand factor. Reviewed

"Matsushita, T. and Sadler, J."

J. Biol. Chem. Vol. 270 ( 22 ) page： 13406-13414 1995

Molecular mechanism and classification of von Willebrand disease Reviewed

"Sadler, J. E., Matsushita, T., Dong, Z., Tuley, E. and Westfield, L. A."

Thromb. Haemost. Vol. 74 ( 1 ) page： 161-166 1995

Diagnosis of hemophilia B carriers using two novel dinucleotide polymorphisms and Hha I RFLP of the factor IX gene in Japanese subjects Reviewed

"Toyozumi, H., Kojima, T., Matsushita, T., Hamaguchi, M., Tanimoto, M. and Saito, H."

Thrombosis & Haemostasis Vol. 74 ( 4 ) page： 1009-1014 1995

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

"We identified two novel dinucleotide polymorphisms in intron A at nucleotide 192 (FIX192) and in the 5' flanking region at nucleotide -793 (FIX-793) of the factor IX gene, which are present in normal Japanese. The Hha I restriction fragment length polymorphism (FIX-HhaI) located 8 kb 3' to the factor IX gene was also found to be an efficient marker for detecting carriers in a Japanese family with hemophilia B. Each of these polymorphisms was able to be rapidly ascertained by the polymerase chain reaction (PCR) technique. In 22 Japanese families with hemophilia B, 18 families (81.8%) were heterozygous for at least one of these polymorphisms, whereas 11 (50%) were informative for the extragenic DXS99/Sac I RFLP which was previously reported as a useful gene marker for Japanese hemophilia B. Using all 4 polymorphisms together, the informative rate improved to 86.4%. Carrier detection and, possibly, the prenatal diagnosis of hemophilia B can be achieved effectively and rapidly in Japanese with these polymorphisms."

Dominant type 1 von Willebrand disease caused by mutated cysteine residues in the D3 domain of von Willebrand factor. Reviewed

"Eikenboom, J. C. J., Matsushita, T., Reitsma, P. H., Tuley, E. A., Castman, G., Briテゥt, E. and Sadler, J. E."

Blood Vol. 88 ( 7 ) page： 2433-2441 1996

Identification of the five hydrophilic residues (Lys-217, Lys-218, Arg- 359, His-360, and Arg-513) essential for the structure and activity of vitamin K-dependent carboxylase Reviewed

"Shimizu, A., Sugiura, I., Matsushita, T., Kojima, T., Hirai, M. and Saito, H."

Biochem Biophys Res Commun Vol. 251 ( 1 ) page： 16285-94 1998

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

"Vitamin K-dependent carboxylase catalyzes the posttranslational conversion of glutamic acid to gamma-carboxyglutamic acid in vitamin K- dependent proteins. The clustered charged-to-alanine scanning mutagenesis of bovine carboxylase has identified five distinct candidate regions (I. Sugiura et al., J. Biol. Chem. 271, 17837-17844, 1996) with significant loss-of-function phenotype. To further specify the residues essential for the structure and function of the enzyme, Lys-217, Lys-218, Arg-359, His-360, Lys-361, Arg-513, and Lys-515 were analyzed by substituting to alanine individually. All the mutants except for K217A were expressed in Chinese hamster ovary cells. The carboxylase activities of R359A, H360A, and R513A decreased in parallel with the vitamin K epoxidase activities. Both carboxylations by R359A and H360A were stimulated saturatively at 1 microM factor IX propeptide (proFIX18) concentration, but that by R513A was not at a concentration up to 128 microM. K218A completely lost the enzyme activities but it cross-linked to the propeptide, suggesting that Lys-218 is critical for enzyme activity without affecting propeptide binding. We conclude that Lys-218, Arg-359, and His-360 are involved in the catalytic event, and Arg-513 participates in propeptide binding. Copyright 1998 Academic Press."

ERGIC-53 Gene Structure and Mutation Analysis in 19 Combined Factors V and VIII Deficiency Families Reviewed

"Nichols, W. C., Terry, V. H., Wheatley, M. A., Yang, A., Zivelin, A., Ciavarella, N., Stefanile, C., Matsushita, T., Saito, H., de Bosch, N. B., Ruiz-Saez, A., Torres, A., Thompson, A. R., Feinstein, D. I., White, G. C., Negrier, C., Vinciguerra, C., Aktan, M., Kaufman, R. J., Ginsburg, D. and Seligsohn, U."

Blood Vol. 93 ( 7 ) page： 2261-2266 1999

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

"Combined factors V and VIII deficiency is an autosomal recessive bleeding disorder associated with plasma levels of coagulation factors V and VIII approximately 5% to 30% of normal. The disease gene was recently identified as the endoplasmic reticulum-Golgi intermediate compartment protein ERGIC-53 by positional cloning, with the detection of two founder mutations in 10 Jewish families. To identify mutations in additional families, the structure of the ERGIC-53 gene was determined by genomic polymerase chain reaction (PCR) and sequence analysis of bacterial artificial chromosome clones containing the ERGIC- 53 gene. Nineteen additional families were analyzed by direct sequence analysis of the entire coding region and the intron/exon junctions. Seven novel mutations were identified in 10 families, with one additional family found to harbor one of the two previously described mutations. All of the identified mutations would be predicted to result in complete absence of functional ERGIC-53 protein. In 8 of 19 families, no mutation was identified. Genotyping data indicate that at least two of these families are not linked to the ERGIC-53 locus. Taken together, these results suggest that a significant subset of combined factors V and VIII deficiency is due to mutation in one or more additional genes."

Severe factor VII deficiency caused by a novel mutation His348 to Gln in the catalytic domain Reviewed

"Katsumi, A., Matsushita, T., Yamazaki, T., Sugiura, I., Kojima, T. and Saito, H."

Thromb Haemost Vol. 83 ( 2 ) page： 239-43 2000

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

"Factor VII is a vitamin K-dependent zymogen that plays a key role in the initiation of the extrinsic pathway. A severe factor VII deficiency was identified in a 45-year old male whose plasma factor VII antigen was less than 60 ng/ml and expressed 5.2% of normal factor VII activity. DNA sequence analysis of the patient's factor VII gene showed a thymidine to guanine transversion at nucleotide 10968 in exon VIII that results in a novel amino acid substitution of His348 to Gln. The patient was homozygous for this mutation, whereas some of his family members were heterozygous. Both wild type and mutant factor VII were transiently expressed in COS-1 cells. The level of secreted mutant factor VII antigen was only 11.0% of the level of wild type factor VII. In CHO cells stably transfected with the mutant factor VII, only 37.3% of the total labeled FVII was secreted into the conditioned media and the remainder was retained inside the cells. These data suggest this mutation leads to factor VII deficiency due to the impaired secretion of the molecule."

Complete antithrombin deficiency in mice results in embryonic lethality Reviewed

"Ishiguro, K., Kojima, T., Kadomatsu, K., Nakayama, Y., Takagi, A., Suzuki, M., Takeda, N., Ito, M., Yamamoto, K., Matsushita, T., Kusugami, K., Muramatsu, T. and Saito, H."

J Clin Invest Vol. 106 ( 7 ) page： 873-878 2000

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

"Antithrombin is a plasma protease inhibitor that inhibits thrombin and contributes to the maintenance of blood fluidity. Using targeted gene disruption, we investigated the role of antithrombin in embryogenesis. Mating mice heterozygous for antithrombin gene (ATIII) disruption, ATIII(+/-), yielded the expected Mendelian distribution of genotypes until 14.5 gestational days (gd). However, approximately 70% of the ATIII(-/-) embryos at 15.5 gd and 100% at 16.5 gd had died and showed extensive subcutaneous hemorrhage. Histological examination of those embryos revealed extensive fibrin(ogen) deposition in the myocardium and liver, but not in the brain or lung. Furthermore, no apparent fibrin(ogen) deposition was detected in the extensive hemorrhagic region, suggesting that fibrinogen might be decreased due to consumptive coagulopathy and/or liver dysfunction. These findings suggest that antithrombin is essential for embryonic survival and that it plays an important role in regulation of blood coagulation in the myocardium and liver."

Short report: A case of purpura fulminans is caused by homozygous delta8857 mutation (protein C-nagoya) and successfully treated with activated protein C concentrate Reviewed

"Nakayama, T., Matsushita, T., Hidano, H., Suzuki, C., Hamaguchi, M., Kojima, T. and Saito, H."

Br J Haematol Vol. 110 ( 3 ) page： 727-30 2000

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

"We report a Japanese patient who developed purpura fulminans and disseminated intravascular coagulation (DIC) shortly after birth. The patient was diagnosed to be homozygous for protein C deficiency and was treated with an activated protein C (APC) concentrate. Intravenous infusions of APC markedly improved the necrotic skin lesions and the anticoagulation by APC enabled successful DIC control. The identified mutation (Delta8857) results in impaired intracellular transport and protein maturation and would be the cause of the complete protein C deficiency. This is the seventh case of the mutation that has been exclusively reported in Japan, but is the first report of a homozygous case. Our findings propose new therapeutic and diagnostic tools for the management of this fatal thrombotic disease."

Identification of plasma antibody epitopes and gene abnormalities in Japanese hemophilia A patients with factor VIII inhibitor Reviewed

"Sugihara, T., Takahashi, I., Kojima, T., Okamoto, Y., Yamamoto, K., Kamiya, T., Matsushita, T. and Saito, H."

Nagoya J Med Sci Vol. 63 page： 25-39 2000

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

"Eleven Japanese hemophilia A patients with anti-factor VIII (FVIII) inhibitors were studied to localize both their inhibitory antibody epitopes and their genotypes. The inhibitor epitopes were studied in nine severe hemophilia A patients by means of a scanning method using the oligopeptide panel covering the FVIII polypeptides without the B domain. The 107 15 mer-peptides were synthesized on solid-phase pins and analyzed for their reactivity with diluted patient plasma. As indicated previously, a series of peptides corresponding to the A2 and C2 domains were recognized by plasma antibodies from 2 patients and 4 patients, respectively. In contrast, all the antibodies bound to several epitopes in the A3 domain, while an epitope 1809-1821 covering the putative factor IX binding site was found in 3 patients. Southern blotting analysis showed that 8 out of 11 patients had either gene deletions or inversions of the FVIII gene, indicating a higher proportion of gross gene alterations in inhibitor-positive hemophilia A patients. However, the correlation of gene abnormality type with epitope location was not fully established."

DNA sequence analysis of protein S deficiency--identification of four point mutations in twelve Japanese subjects Reviewed

"Iwaki, T., Mastushita, T., Kobayashi, T., Yamamoto, Y., Nomura, Y., Kagami, K., Nakayama, T., Sugiura, I., Kojima, T., Takamatsu, J., Kanayama, N. and Saito, H."

Semin Thromb Hemost Vol. 27 ( 2 ) page： 155-60 2001

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

"The molecular basis for the hereditary type I protein S (PS) deficiency was investigated. DNA sequence analysis of 12 patients with PS deficiency in Japan identified four point mutations and three of them were novel. Nonsense mutations found in two unrelated patients resulted in termination of the PS polypeptide chains at Gln 238 and Lys 392, respectively. Two novel missense mutations were also found in two other patients substituting Asp 202 for Asn and Leu 298 for Pro, respectively. Comparison of the PS amino acid sequences from several mammalians indicated that Asp 202 and Leu 298 were preserved and thus appeared to be responsible for the pathogenesis of PS deficiency."

Localization of von willebrand factor-binding sites for platelet glycoprotein Ib and botrocetin by charged-to-alanine scanning mutagenesis Reviewed

Matsushita, T., Meyer, D. and Sadler, J. E.

J Biol Chem Vol. 275 ( 15 ) page： 11044-9 2000

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

At sites of vascular injury, von Willebrand factor (VWF) mediates platelet adhesion through binding to platelet glycoprotein Ib (GPIb). Previous studies identified clusters of charged residues within VWF domain A1 that were involved in binding GPIb or botrocetin. The contribution of 28 specific residues within these clusters was analyzed by mutating single amino acids to alanine. Binding to a panel of six conformation-dependent monoclonal antibodies was decreased by mutations at Asp(514), Asp(520), Arg(552), and Arg(611) (numbered from the N- terminal Ser of the mature processed VWF), suggesting that these residues are necessary for domain A1 folding. Binding of (125)I- botrocetin was decreased by mutations at Arg(629), Arg(632), Arg(636), and Lys(667). Ristocetin-induced and botrocetin-induced binding to GPIb both were decreased by mutations at Lys(599), Arg(629), and Arg(632); among this group the K599A mutant was unique because (125)I-botrocetin binding was normal, suggesting that Lys(599) interacts directly with GPIb. Ristocetin and botrocetin actions on VWF were dissociated readily by mutagenesis. Ristocetin-induced binding to GPIb was reduced selectively by substitutions at positions Lys(534), Arg(571), Lys(572), Glu(596), Glu(613), Arg(616), Glu(626), and Lys(642), whereas botrocetin-induced binding to GPIb was decreased selectively by mutations at Arg(636) and Lys(667). The binding of monoclonal antibody B724 involved Lys(660) and Arg(663), and this antibody inhibits (125)I- botrocetin binding to VWF. The crystal structure of the A1 domain suggests that the botrocetin-binding site overlaps the monoclonal antibody B724 epitope on helix 5 and spans helices 4 and 5. The binding of botrocetin also activates the nearby VWF-binding site for GPIb that involves Lys(599) on helix 3.

Mutations in the NMMHC-A gene cause autosomal dominant macrothrombocytopenia with leukocyte inclusions (May-Hegglin anomaly/Sebastian syndrome) Reviewed

"Kunishima, S., Kojima, T., Matsushita, T., Tanaka, T., Tsurusawa, M., Furukawa, Y., Nakamura, Y., Okamura, T., Amemiya, N., Nakayama, T., Kamiya, T. and Saito, H."

Blood Vol. 97 ( 4 ) page： 1147-9. 2001

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

"Macrothrombocytopenia with leukocyte inclusions is a rare autosomal dominant platelet disorder characterized by a triad of giant platelets, thrombocytopenia, and characteristic Dohle body-like leukocyte inclusions. A previous study mapped a locus for the disease on chromosome 22q12.3-q13.2 by genome-wide linkage analysis. In addition, the complete DNA sequence of human chromosome 22 allowed a positional candidate approach, and results here indicate that the gene encoding nonmuscle myosin heavy chain-A, NMMHC-A, is mutated in this disorder. Mutations were found in 6 of 7 Japanese families studied: 3 missense mutations, a nonsense mutation, and a one-base deletion resulting in a premature termination. Immunofluorescence studies revealed that NMMHC-A distribution in neutrophils appeared to mimic the inclusion bodies. These results provide evidence for the involvement of abnormal NMMHC-A in the formation of leukocyte inclusions and also in platelet morphogenesis."

Cloning and characterization of the murine antithrombin gene Reviewed

"Nakayama, Y., Kojima, T., Takagi, A., Yanada, M., Yamamoto, K., Matsushita, T. and Saito, H."

Thromb Res Vol. 100 ( 3 ) page： 179-83 2000

Syndecan-4 deficiency leads to high mortality of lipopolysaccharide- injected mice Reviewed

"Ishiguro, K., Kadomatsu, K., Kojima, T., Muramatsu, H., Iwase, M., Yoshikai, Y., Yanada, M., Yamamoto, K., Matsushita, T., Nishimura, M., Kusugami, K., Saito, H. and Muramatsu, T."

J Biol Chem Vol. 3 page： 3 2001

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

"Syndecan-4 is a transmembrane heparan sulfate proteoglycan belonging to the syndecan family. Following intraperitoneal injection of lipopolysaccharide (LPS), syndecan-4-deficient mice exhibited high mortality compared to wild-type controls. Severe endotoxin shock was observed in the deficient mice: systolic blood pressure and left ventricular fractional shortening were lower in the deficient mice than in the wild-type controls 9 h after LPS injection. Although histological examinations revealed no apparent differences between two groups, the plasma level of interleukin (IL)-1b was higher in the deficient mice than in the wild-type controls 9 h after LPS injection. Consistent with the regulatory roles of syndecan-4, its expression in monocytes and endothelial cells of microvasculature increased in the wild-type mice after LPS administration. Although IL-1b was produced to the same extent by macrophages from syndecan-4-deficient and wild-type mice after LPS stimulation, inhibition of its production by transforming growth factor (TGF)-b1 was impaired in the syndecan-4- deficient macrophages. These results indicate that syndecan-4 could be involved in prevention of endotoxin shock, at least partly through the inhibitory action of TGF-b1 on IL-1b production."

Plasma levels of syndecan-4 (ryudocan) are elevated in patients with acute myocardial infarction Reviewed

"Kojima, T., Takagi, A., Maeda, M., Segawa, T., Shimizu, A., Yamamoto, K., Matsushita, T. and Saito, H."

Thromb Haemost Vol. 85 ( 5 ) page： 793-9 2001

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

"Syndecan-4 (ryudocan) is a cell-surface heparan sulfate proteoglycan, which plays an important role in a variety of biological functions including regulation of blood coagulation. cell adhesion, and cell growth. In this study, we measured plasma levels of syndecan-4 in patients with acute myocardial infarction using an enzyme-immunoassay, and found that they were extremely high, with a peak of average (10.5 +/- 5.6 ng/ml, 2 weeks after onset), as compared with those in normal subjects (0.078 +/- 0.030 ng/ml) (p < 0.001). We also observed a distinct expression of syndecan-4 in the repair region of the damaged cardiac tissues with infarction, but not in intact region, by immunohistochemical analysis. To clarify the mechanism of syndecan-4 induction, we investigated the hypoxia effect on its expression, and found that hypoxia treatment up-regulated the gene expression of syndecan-4 in various types of cells. Taken together, it is suggested that syndecan-4 is induced by hypoxia stimuli in ischemic heart tissues, and may function as a tissue-repair molecule via biological mediators such as heparin-binding growth factors."

Identification of six novel MYH9 mutations and genotype-phenotype relationships in autosomal dominant macrothrombocytopenia with leukocyte inclusions Reviewed

"Kunishima, S., Matsushita, T., Kojima, T., Amemiya, N., Choi, Y. M., Hosaka, N., Inoue, M., Jung, Y., Mamiya, S., Matsumoto, K., Miyajima, Y., Zhang, G., Ruan, C., Saito, K., Song, K. S., Yoon, H. J., Kamiya, T. and Saito, H."

J Hum Genet Vol. 46 ( 12 ) page： 722-9 2001

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

"The autosomal dominant macrothrombocytopenia with leukocyte inclusions, May-Hegglin anomaly (MHA), Sebastian syndrome (SBS), and Fechtner syndrome (FTNS), are rare platelet disorders characterized by a triad of giant platelets, thrombocytopenia, and characteristic Dohle body-like leukocyte inclusions. The locus for these disorders was previously mapped on chromosome 22q12.3-q13.2 and the disease gene was recently identified as MYH9, the gene encoding the nonmuscle myosin heavy chain-A. To elucidate the spectrum of MYH9 mutations responsible for the disorders and to investigate genotypephenotype correlation, we examined MYH9 mutations in an additional 11 families and 3 sporadic patients with the disorders from Japan. Korea, and China. All 14 patients had heterozygous MYH9 mutations, including three known mutations and six novel mutations (three missense and three deletion mutations). Two cases had Alport manifestations including deafness, nephritis, and cataracts and had R1165C and E1841K mutations, respectively. However, taken together with three previous reports, including ours, the data do not show clear phenotype-genotype relationships. Thus, MHA, SBS, and FTNS appear to represent a class of allelic disorders with variable phenotypic diversity."

"Binding site on human von Willebrand factor of bitiscetin, a snake venom-derived platelet aggregation inducer" Reviewed

"Matsui, T., Hamako, J., Matsushita, T., Nakayama, T., Fujimura, Y. and Titani, K."

Biochemistry Vol. 41 ( 25 ) page： 7939-46. 2002

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

"Bitiscetin, a C-type lectin-like heterodimeric snake venom protein purified from Bitis arietans, binds to human von Willebrand factor (VWF) and induces the platelet membrane glycoprotein (GP) Ib-dependent platelet agglutination in vitro similar to botrocetin. In contrast with botrocetin which binds to the A1 domain of VWF, the A3 domain, a major collagen-binding site of VWF, was proposed to be a bitiscetin-binding site. In the competitive binding assay, neither bitiscetin nor botrocetin had an inhibitory effect on the VWF binding to the immobilized type III collagen on a plastic plate. The anti-VWF monoclonal antibody NMC-4, which inhibits VWF-induced platelet aggregation by binding to alpha4 helix of the A1 domain, also inhibited bitiscetin binding to the VWF. Binding of VWF to the immobilized bitiscetin was competitively inhibited by a high concentration of botrocetin. A panel of recombinant VWF, in which alanine-scanning mutagenesis was introduced to the charged amino acid residues in the A1 domain, showed that the bitiscetin-binding activity was reduced in mutations at Arg632, Lys660, Glu666, and Lys673 of the A1 domain. Those substituted at Arg629, Arg636, and Lys667, which decreased the botrocetin binding, showed no effect on the bitiscetin binding. These results indicate that bitiscetin binds to a distinct site in the A1 domain of VWF spanning over alpha4a, alpha5 helices and the loop between alpha5 and beta6 but close to the botrocetin- and NMC-4-binding sites. Monoclonal antibodies recognizing the alpha-subunit of bitiscetin specifically inhibited bitiscetin-induced platelet agglutination without affecting the binding between VWF and bitiscetin, suggesting that the alpha-subunit of bitiscetin is located on VWF closer to the GPIb-binding site than the beta-subunit is. Bitiscetin and botrocetin might modulate VWF by binding to the homologous region of the A1 domain to induce a conformational change leading to an increased accessibility to platelet GPIb."

Identification of the regulatory elements of the human von Willebrand factor for binding to platelet GPIb. Importance of structural integrity of the regions flanked by the CYS1272-CYS1458 disulfide bond Reviewed

"Nakayama, T., Matsushita, T., Dong, Z., Sadler, J. E., Jorieux, S., Mazurier, C., Meyer, D., Kojima, T. and Saito, H."

J Biol Chem Vol. 277 ( 24 ) page： 22063-72. 2002

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

"In vitro platelet glycoprotein Ib (GPIb) binding of the human von Willebrand factor (VWF) increases markedly by exogenous modulators such as ristocetin or botrocetin, and the binding does not occur in normal circulation. GPIb binding sites have been assigned in the VWF A1 domain, which consists of a disulfide loop Cys1272(509)-Cys1458(695) where amino acid residues are numbered from the starting methionine as +1. The previous numbering from the N-terminal Ser of the mature processed VWF is indicated in parentheses. In contrast, several gain-of- function mutations have been found in two regions comprised of the disulfide loop and its N- and C-terminal flanking regions. In this study, Cys1222(459)-Tyr1271(508), Gln1238(475)-Tyr1271(508), Glu1260(497)-Tyr1271(508), and Asp1459(696)-Asp1472(709) were sequentially deleted of full-length multimeric recombinant VWF. Deletions at either side resulted in normal GPIb binding, indicating that the flanking regions are not GPIb binding sites. However, the addition of a mutation at Arg1308(545) on each deletion mutant resulted in spontaneous GPIb binding without requiring modulators, suggesting that both regions are important for the inhibition of GPIb binding. Spontaneous binding was completely inhibited by monoclonal antibodies that recognize the GPIb binding sites. Interestingly, mutant proteins with N-terminal but not C-terminal deletions lost binding to monoclonal antibodies B328, B710, and 23C7, which selectively inhibit ristocetin- induced GPI binding. Their epitopes were found at His1268(505) or Asp1269(506). The crystallographic structure of the A1 domain suggests that GPIb binding is influenced by the molecular interface between the two regions and that the antibody binding to the interface inhibits binding."

Impact of antithrombin deficiency in thrombogenesis: lipopolysaccharide and stress-induced thrombus formation in heterozygous antithrombin- deficient mice Reviewed

"Yanada, M., Kojima, T., Ishiguro, K., Nakayama, Y., Yamamoto, K., Matsushita, T., Kadomatsu, K., Nishimura, M., Muramatsu, T. and Saito, H."

Blood Vol. 99 ( 7 ) page： 2455-8. 2002

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

"Antithrombin (AT) deficiency is an autosomal disorder associated with venous thromboembolism. However, a diagnosis of homozygous AT deficiency is seldom made. Most patients are heterozygous and have approximately 50% AT activities, and they are at higher risk for the development of thromboembolism. Through gene targeting we generated AT- deficient mice and previously reported that completely AT-deficient mice could not survive the prenatal period because of extensive thrombosis in the myocardium and liver sinusoids. In contrast, heterozygous AT-deficient mice with 50% AT activities have not shown spontaneous thromboembolic episodes. To demonstrate a thrombotic tendency in heterozygous AT deficiency, we challenged heterozygous AT- deficient mice (AT+/- mice) with the administration of lipopolysaccharide (LPS) or with restraint stress by immobilization. LPS injection markedly induced fibrin deposition in the kidney glomeruli, myocardium, and liver sinusoids in AT+/- mice compared with wild-type mice (AT+/+ mice). Restraint stress tests were performed by placing mice in 50-mL conical centrifuge tubes for 20 hours. Fibrin deposition was observed in the kidney of AT+/+ and AT+/- mice, but AT+/- mice exhibited more extensive fibrin deposition than AT+/+ mice. After prophylactic administration of human AT concentrates to increase plasma AT activities of AT+/- mice, LPS-induced fibrin deposition was effectively prevented. These results suggest that heterozygous AT deficiency is significantly associated with a tendency toward thrombosis formation in the kidney. The AT+/- mouse thus is a useful model for studying the effect of environmental or genetic risk factors on thrombogenesis."

Increased expression of plasminogen activator inhibitor-1 with fibrin deposition in a murine model of aging, ""Klotho"" mouse Reviewed

"Takeshita, K., Yamamoto, K., Ito, M., Kondo, T., Matsushita, T., Hirai, M., Kojima, T., Nishimura, M., Nabeshima, Y., Loskutoff, D. J., Saito, H. and Murohara, T.

Semin Thromb Hemost Vol. 28 ( 6 ) page： 545-54 2002

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

Although aging accompanies specific pathological changes, including thrombosis and organ sclerosis, the underlying mechanisms of these processes remain to be elucidated. In the present study, we analyzed the gene expression of plasminogen activator inhibitor-1 (PAI-1), a key molecule in the development of thrombosis, in a murine model of aging, klotho mutant (kl/kl) mice. Active PAI-1 antigen in plasma and PAI-1 mRNA in several tissues were strikingly elevated in kl/kl mice as compared with wild-type mice. This increased PAI-1 expression was age dependent and linked to the development of ectopic calcification and glomerular fibrin deposition in the kidneys. In situ hybridization analysis of kl/kl mice demonstrated that strong signals for PAI-1 mRNA were localized in renal tubular epithelial cells, cardiomyocytes, adrenal medullar cells, and smooth muscle and endothelial cells in Monckeberg's arteriosclerotic vessels. Renal glomerular fibrin deposition, as evaluated immunohistochemically, was occasionally observed only in kl/kl mice, and the number of fibrin-positive glomeruli increased as the kl/kl mice aged. These observations suggest that in the process of aging the PAI-1 gene expression is increased, contributing to the development of thrombosis.

Novel nonsense mutation in the platelet glycoprotein Ibbeta gene associated with Bernard-Soulier syndrome Reviewed

"Kunishima, S., Matsushita, T., Ito, T., Kamiya, T. and Saito, H."

Am J Hematol Vol. 71 ( 4 ) page： 279-84 2002

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

"Bernard-Soulier syndrome (BSS) is an autosomal recessive bleeding disorder caused by quantitative or qualitative abnormalities in the glycoprotein (GP) Ib/IX/V complex, the platelet receptor for von Willebrand factor. This complex is composed of four subunits, GPIbalpha, GPIbbeta, GPIX, and GPV, and the coordinated assembly of GPIbalpha, GPIbbeta, and GPIX is required for the efficient surface expression of a functional complex. We report here a novel nonsense mutation of the GPIbbeta gene associated with BSS. Flow cytometric analysis of the patient's platelets showed markedly reduced GPIbalpha and absent GPIX surface expression. Immunoblot analysis of solubilized platelets showed that a small amount of GPIbalpha was detected; however, GPIbbeta and GPIX were undetectable. DNA sequencing analysis revealed a novel nonsense mutation of the GPIbbeta gene that converts Trp (TGG) to a stop codon (TAG) at residue 123. Transient transfection studies revealed that the mutant GPIbbeta polypeptide was not detected in the transfected 293T cells, suggesting that null expression of the mutant GPIbbeta impairs expression of the GPIbalpha and GPIX subunits and results in a BSS phenotype in the patient."

Immunofluorescence analysis of neutrophil nonmuscle myosin heavy chain-A in MYH9 disorders: association of subcellular localization with MYH9 mutations Reviewed

"Kunishima, S., Matsushita, T., Kojima, T., Sako, M., Kimura, F., Jo, E. K., Inoue, C., Kamiya, T. and Saito, H."

Lab Invest Vol. 83 ( 1 ) page： 115-22 2003

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

"In this study, a total of 24 cases with MYH9 disorders and suspected cases were subjected to immunofluorescence analysis by a polyclonal antibody against human platelet NMMHCA. Abnormal subcellular localization of NMMHCA was observed in every neutrophil from individuals with MYH9 mutations. Comparison with May-Grunwald-Giemsa staining revealed that the NMMHCA always coexisted with the neutrophil inclusion bodies, suggesting that NMMHCA is associated with such bodies. In three cases, neutrophil inclusions were not detected on conventional May-Grunwald-Giemsa-stained blood smears but immunofluorescence analysis revealed the abnormal NMMHCA localization. In contrast, cases with Epstein syndrome and the isolated macrothrombocytopenia with normal NMMHCA localization had no MYH9 mutations. An antibody that recognizes the C-terminal 12 mer peptides showed similar immunoreactivity from the patients heterozygous for truncated mutations that abolished the C-terminal epitope, suggesting that normal NMMHCA dimerizes with abnormal NMMHCA to form inclusion bodies. We further propose that the localization pattern can be classified into three groups according to the number, size, and shape of the fluorescence-labeled NMMHCA granule. Immunofluorescence analysis of neutrophil NMMHCA is useful as a screening test for the clear hematopathologic classification of MYH9 disorders."

Compound heterozygosity for two novel mutations in a severe factor XI deficiency Reviewed

"Tsukahara, A., Yamada, T., Takagi, A., Murate, T., Matsushita, T., Saito, H. and Kojima, T."

Am J Hematol Vol. 73 ( 4 ) page： 279-84 2003

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

"We identified two novel mutations in an asymptomatic 25-year-old Japanese patient with severe factor XI deficiency. Direct sequencing analysis of PCR products from his factor XI gene revealed a G to T transversion in exon 12, resulting in the nonsense mutation (Glu447Stop) and a G insertion in five consecutive guanine nucleotides ((501)Trp(TGG)-(502)Gly(GGG)) in exon 13 that is expected to lead to the substitution of the last 105 amino acids ((503)Tyr-(607)Val) with 32 abnormal amino acid residues ((503)Val-(534)Thr) followed by stop codon. We also demonstrated that two mutations are associated with the separate alleles in this patient, indicating compound heterozygosity for these mutations. Both mutations lead to the disruption of the catalytic domain structure of the FXI molecule and thus are responsible for his deficiency of factor XI."

"Enzyme immunoassay for measurement of murine plasminogen activator inhibitor-1, employing a specific antibody produced by the DNA vaccine method" Reviewed

"Yamada, T., Takagi, A., Takeshita, K., Yamamoto, K., Ito, M., Matsushita, T., Murate, T., Saito, H. and Kojima, T."

Thromb Res Vol. 111 page： 285-91 2003

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

"We developed a sensitive immunoassay to determine the concentration of mouse plasminogen activator inhibitor-1. The assay was a non-competitive sandwich enzyme-linked immunosorbent assay (ELISA) based on the production of a specific polyclonal antibody against mouse plasminogen activator inhibitor type-1 (PAI-1) used both as a trapping and detecting antibody. This antibody was raised in a rabbit by direct introduction of the expression vector plasmid DNA encoding mouse PAI-1, instead of conventional immunization with the purified protein. The standard curve was constructed with a recombinant glutathione S-transferase (GST)-mouse PAI-1 fusion protein (GST-mPAI-1) and dose-response of the assay was linear for GST-mPAI-1 between 6.25 and 100 pM. In order to assess the consistency of the assay, we measured PAI-1 antigen in normal mouse pooled plasma several times. We found that the intra-assay and inter-assay coefficients of variation (CV) were 4.8% and 9.2%, respectively, indicating that the ELISA would be sufficiently repeatable and reproducible. In this assay, lipopolysaccharide (LPS)-injected mice showed substantially higher levels (22-fold) of plasma PAI-1 antigen than did control mice (12.5+/-2.4 vs. 0.58+/-0.16 nM), similar to results reported elsewhere. Taken together, the DNA vaccine method is extremely useful for preparing specific antibodies against mouse PAI-1, which can be utilized to establish the ELISA and analyze the profile of PAI-1 distributions in mice under various conditions. This approach might also be useful for immunological investigation of other coagulation factors and related proteins."

*Identification of the Amino Acid Residues of the Platelet Glycoprotein Ib (GPIb) Essential for the von Willebrand Factor Binding by Clustered Charged-to-Alanine Scanning Mutagenesis Reviewed

"Shimizu, A., Matsushita, T., Kondo, T., Inden, Y., Kojima, T., Saito, H. and Hirai, M."

J Biol Chem Vol. 279 ( 16 ) page： 16285-94 2004

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

At the site of vascular injury, von Willebrand factor (VWF) mediates platelet adhesion to subendothelial connective tissue through binding to the N-terminal domain of the alpha chain of platelet glycoprotein Ib (GPIbalpha). To elucidate the molecular mechanisms of the binding, we have employed charged-to-alanine scanning mutagenesis of the soluble fragment containing the N-terminal 287 amino acids of GPIbalpha. Sixty-two charged amino acids were changed singly or in small clusters, and 38 mutant constructs were expressed in the supernatant of 293T cells. Each mutant was assayed for binding to several monoclonal antibodies for human GPIbalpha and for ristocetin-induced and botrocetin-induced binding of (125)I-labeled human VWF. Mutations at Glu(128), Glu(172), and Asp(175) specifically decreased both ristocetin- and botrocetin-induced VWF binding, suggesting that these sites are important for VWF binding of platelet GPIb. Monoclonal antibody 6D1 inhibited ristocetin- and botrocetin-induced VWF binding, and a mutation at Glu(125) specifically reduced the binding to 6D1. In contrast, antibody HPL7 had no effect for VWF binding, and mutant E121A reduced the HPL7 binding. Mutations at His(12) and Glu(14) decreased the ristocetin-induced VWF binding with normal botrocetin-induced binding. Crystallographic modeling of the VWF-GPIbalpha complex indicated that Glu(128) and Asp(175) form VWF binding sites; the binding of 6D1 to Glu(125) interrupts the VWF binding of Glu(128), but HPL7 binding to Glu(121) has no effect on VWF binding. Moreover, His(12) and Glu(14) contact with Glu(613) and Arg(571) of VWF A1 domain, whose mutations had shown similar phenotype. These findings indicated the novel binding sites required for VWF binding of human GPIbalpha.

Identification of protein Salpha gene mutations including four novel mutations in eight unrelated patients with protein S deficiency Reviewed

"Okada, H., Takagi, A., Murate, T., Adachi, T., Yamamoto, K., Matsushita, T., Takamatsu, J., Sugita, K., Sugimoto, M., Yoshioka, A., Yamazaki, T., Saito, H. and Kojima, T."

Br J Haematol Vol. 126 ( 2 ) page： 219-25 2004

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

"Eight distinct and potentially causative mutations were identified in eight unrelated Japanese patients with protein S (PS) deficiency, by direct DNA sequencing of the protein Salpha (PSalpha) gene-specific polymerase chain reaction products of all 15 exons and exon/intron boundaries. There were five missense mutations, including two novel mutations (Cys80Tyr and Arg314His), and three showed a major impact on the expected gene products: novel mutations of a 5-bp deletion (delCTCTG887:Cys206Stop) and a nonsense mutation (Glu208Stop), as well as a previously reported splice site (exon 10 +5 A-->G) mutation. One of the patients showed compound heterozygosity for delCTCTG887 and 732A-->G. Investigation for the cosegregation state of these two mutations with PS deficiency in the patient's family suggested that the delCTCTG887 mutation was responsible for the abnormal phenotype and that the 732A-->G (Lys155Glu) mutation did not appear to play a key role. However, we also identified the same 732A-->G (Lys155Glu) mutation in an unrelated patient with apparent PS deficiency with severe pulmonary embolism, and found that this mutation seemed to cosegregate with a PS-deficient state in her family members. These data implied that unknown factor(s) other than the 732A-->G mutation itself might influence phenotypic expression of PS status in different individuals."

*Targeted disruption of mouse ortholog of the human MYH9 responsible for macrothrombocytopenia with different organ involvement: hematological, nephrological, and otological studies of heterozygous KO mice Reviewed

Matsushita, T., Hayashi, H., Kunishima, S., Hayashi, M., Ikejiri, M., Takeshita, K., Yuzawa, Y., Adachi, T., Hirashima, K., Sone, M., Yamamoto, K., Takagi, A., Katsumi, A., Kawai, K., Nezu, T., Takahashi, M., Nakashima, T., Naoe, T., Kojima, T. and Saito, H.

Biochem Biophys Res Commun Vol. 325 ( 4 ) page： 1163-71 2004

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

Among three different isoforms of non-muscle myosin heavy chains (NMMHCs), only NMMHCA is associated with inherited human disease, called MYH9 disorders, characterized by macrothrombocytopenia and characteristic granulocyte inclusions. Here targeted gene disruption was performed to understand fundamental as well as pathological role of the gene for NMMHCA, MYH9. Heterozygous intercrosses yielded no homozygous animals among 552 births, suggesting that MYH9 expression is required for embryonic development. In contrast, MYH9+/- mice were viable and fertile without gross anatomical, hematological, and nephrological abnormalities. Immunofluorescence analysis also showed the normal cytoplasmic distribution of NMMHCA. We further measured the auditory brainstem response and found two of six MYH9+/- mice had hearing losses, whereas the remaining four were comparable to wild-type mice. Such observation may parallel the diverse expression of Alport's manifestations of human individuals with MYH9 disorders and suggest the limited requirement of the gene for maintenance and function of specific organs.

First description of somatic mosaicism in MYH9 disorders Reviewed

"Kunishima, S., Matsushita, T., Yoshihara, T., Nakase, Y., Yokoi, K., Hamaguchi, M. and Saito, H."

Br J Haematol Vol. 128 ( 3 ) page： 360-5 2005

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

"MYH9 disorders are characterized by giant platelets, thrombocytopenia, and Dohle body-like cytoplasmic granulocyte inclusion bodies that result from mutations in MYH9, which encodes non-muscle myosin heavy chain-A (NMMHCA). These disorders are known to be transmitted in an autosomal dominant manner, although about 20% of cases are considered to be sporadic. We report here the first case of a MYH9 disorder because of somatic mosaicism. The patient was the father of a male with typical May-Hegglin anomaly. The father had normal platelet counts, however, both normal-sized and giant platelets were observed on his peripheral blood smears. In addition, 14% of neutrophils contained inclusion bodies and the rest showed a normal morphology. Quantitative fluorescent polymerase chain reaction analysis showed that only 6% of DNA from peripheral blood leucocytes harboured the mutation. The mosaicism was demonstrated at a similar rate in different tissues, buccal mucosa cells and hair bulb cells, implying that the mutation had occurred before gastrulation. Mosaicism might account for some de novo mutations in MYH9 disorders."

Detection of unique neutrophil non-muscle myosin heavy chain-A localization by immunofluorescence analysis in MYH9 disorder presented with macrothrombocytopenia without leukocyte inclusions and deafness Reviewed

"Kunishima, S., Matsushita, T., Shiratsuchi, M., Ikuta, T., Nishimura, J., Hamaguchi, M., Naoe, T. and Saito, H."

Eur J Haematol Vol. 74 ( 1 ) page： 1-5 2005

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

"MYH9 disorders are autosomal-dominant macrothrombocytopenias with leukocyte inclusions caused by mutations in the MYH9 gene, which encodes the non-muscle myosin heavy chain-A (NMMHCA). We report a patient with an MYH9 disorder who presented with macrothrombocytopenia without leukocyte inclusions and severe bilateral sensory deafness. Conventional May-Grunwald-Giemsa staining failed to detect granulocyte cytoplasmic inclusions, whereas immunofluorescence analysis clearly demonstrated abnormal neutrophil NMMHCA localization. Genetic analyses revealed a novel heterozygous 18 base deletion in MYH9, leading to a six-amino acid in-frame deletion (N76_S81del) in NMMHCA. These results further support the usefulness of immunofluorescence analysis in differential diagnosis of MYH9 disorders."

Obesity enhances the induction of plasminogen activator inhibitor-1 by restraint stress: a possible mechanism of stress-induced renal fibrin deposition in obese mice Reviewed

"Yamamoto, K., Kojima, T., Adachi, T., Hayashi, M., Matsushita, T., Takamatsu, J., Loskutoff, D. J. and Saito, H."

Journal of Thrombosis and Haemostasis Vol. 3 ( 7 ) page： 1495-1502 2005

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

"Summary. Background and objectives: Cardiovascular/thrombotic diseases are frequently induced by a variety of stressors. Obese patients are susceptible to thrombotic diseases associated with stress, but the underlying mechanism is still unknown. We have begun to investigate the expression of a primary inhibitor of fibrinolysis, plasminogen activator inhibitor-1 (PAI-1), in association with tissue thrombosis, using restraint-stressed obese mice. Methods and results: We analyzed the expression of PAI-1 after restraint (immobilization) stress in genetically obese mice in comparison with their lean counterparts. Dramatic increases in PAI-1 antigen in plasma and in tissue extracts were observed in the obese mice exposed to restraint stress. The induction of PAI-1 mRNA by stress in the tissues was also pronounced in the stressed obese mice as compared with the lean mice, especially in the hearts and adipose tissues. In situ hybridization analysis revealed that strong signals for PAI-1 mRNA were localized in the adipocytes, cardiovascular endothelial cells, and renal glomerular cells of the stressed obese mice. Histological examination revealed that renal glomerular fibrin deposition was detected only in the obese mice after 2 h of restraint stress. Conclusions: Obesity enhances the stress-mediated PAI-1 induction in the blood and tissues. This phenomenon may be associated with the increased risk of stress-induced renal fibrin deposition in obese subjects."

Integrin activation and matrix binding mediate cellular responses to mechanical stretch Reviewed

"Katsumi, A., Naoe, T., Matsushita, T., Kaibuchi, K. and Schwartz, M. A."

J Biol Chem Vol. 280 ( 17 ) page： 16546-9 2005

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

"Mechanical tension is a critical determinant of cell growth, differentiation, apoptosis, migration, and development. Integrins have been implicated in sensing force but little is known about how forces are transduced to biochemical signals. We now show that mechanical strain stimulates conformational activation of integrin alphavbeta3 in NIH3T3 cells. Integrin activation is mediated by phosphoinositol 3-kinase and is followed by an increase in integrin binding to extracellular matrix proteins. Mechanical stretch stimulation of JNK was dependent on new integrin binding to extracellular matrix. These data define a molecular mechanism for the role of integrins in mechanotransduction."

Giant platelet syndrome Reviewed

"Saito, H., Matsushita, T., Yamamoto, K., Kojima, T. and Kunishima, S."

Hematology Vol. 10 Suppl 1 page： 41-6 2005

Identification of amino acid residues essential for heparin binding by the A1 domain of human von Willebrand factor Reviewed

"Adachi, T., Matsushita, T., Dong, Z., Katsumi, A., Nakayama, T., Kojima, T., Saito, H., Sadler, J. E. and Naoe, T."

Biochem Biophys Res Commun Vol. 339 ( 4 ) page： 1178-83 2006

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

"Platelet adhesion is mediated by von Willebrand factor (VWF) that binds platelet glycoprotein Ib (GPIb). Previous observations suggested that heparin competitively inhibits the binding of VWF to GPIb and may down-regulate platelet adhesion. We performed charged-to-alanine scanning mutagenesis of domain A1 and studied dose-dependent binding to heparin-Sepharose beads. Mutations at Lys1362 and Arg1395, at which the GPIb binding was markedly decreased, showed 41% and 42% binding, respectively. Clustered mutations in the segments 1332KDRKR1336 and 1405KKKK1408, which have been proposed as heparin binding sequences, showed 72% and 52% binding, respectively. However, single alanine substitutions within these clusters showed normal binding. Our findings suggest that heparin may inhibit the binding of VWF to GPIb by interacting with GPIb binding and interpret why some hemorrhagic complications of heparin therapy are not predictable based on techniques for monitoring the conventional anticoagulant effects of heparin."

*Fatal thrombosis of antithrombin-deficient mice is rescued differently in the heart and liver by intercrossing with low tissue factor mice Reviewed

Hayashi, M., Matsushita, T., Mackman, N., Ito, M., Adachi, T., Katsumi, A., Yamamoto, K., Takeshita, K., Kojima, T., Saito, H., Murohara, T. and Naoe, T.

Journal of Thrombosis and Haemostasis Vol. 4 ( 1 ) page： 177-185 2006

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

Summary. Background: We previously reported that the targeted disruption of murine antithrombin (AT) gene resulted in embryonic lethality before 16.5 gestational days (gd) because of severe cardiac and hepatic thrombosis. Objective and Methods: To investigate the influences of lowered tissue factor (TF) activity upon hypercoagulation of AT-/- embryos, we crossed AT+/- with low TF (mTF-/-hTF+) mice to yield homozygous AT-deficient mice with the extremely low TF activity, that is expressed from the inserted human TF mini gene. Results: AT-/- embryos either with 50% TF (AT-/-mTF+/-hTF+) or with low (~1% TF, AT-/-mTF-/-hTF+) were not born, although the survival was prolonged until 18.5 gd. In both genotypes, histological examination showed disseminated thrombosis in hepatic sinusoidal space or in the portal veins, suggesting that the thrombogenesis caused loss of hepatic blood flow. As in original AT-/-, AT-/-mTF+/-hTF+ showed subcutaneous (s.c.) bleeding and also suffered from the myocardial degeneration apparently because of coronary thrombus formation. However, AT-/-mTF-/-hTF+ had no skin hemorrhage and the thrombosis and degeneration were completely abolished in the heart. Myocardium of adult low TF mice had exhibited fibrosis secondary to hemorrhage; however, it was significantly decreased in low TF mice with AT+/-. Conclusions: Our current model suggests that, in the heart, TF plays an important role in the thrombogenesis and it counterbalances AT-dependent anticoagulation. AT may be a potent anticoagulant during mice development and the activation and subsequent regulation of TF-procoagulant activity take place differently between the liver and the heart. These differences appear to point to local regulatory mechanisms in murine hemostasis.

Anti-HCV agent, ribavirin, elevates the activity of clotting factor VII in patients with hemophilia: a possible mechanism of decreased events of bleeding in patients with hemophilia by ribavirin Reviewed

"Yamamoto, K., Honda, T., Matsushita, T., Kojima, T. and Takamatsu, J."

J Thromb Haemost Vol. 4 ( 2 ) page： 469-70 2006

Abnormal hemostasis tests and bleeding in chronic liver disease: are they related? No, but they need a careful look Invited

Matsushita, T. and Saito, H.

J Thromb Haemost Vol. 4 ( 9 ) page： 2066-7 2006.9

"[Approaches to anemia, thrombocytopenia, and DIC in cancer patients]" Reviewed

"Matsushita, T."

Gan To Kagaku Ryoho Vol. 33 ( 1 ) page： 6-12 2006

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

"Patients with cancer-related anemia have an inadequate Epo response that is further impaired by cancer treatments such as chemotherapy. Significant number of studies have demonstrated that treatment of anemia in cancer patients using recombinant human EPO(rHuEPO, epoetin alfa) significantly increases hemoglobin(Hb) levels,reduces transfusion requirements,and improves quality of life,particularly by relieving fatigue. However,the findings of several studies have raised the possibility of an adverse effect of thromboembolism. The American Society of Clinical Oncology and the American Society of Hematology developed an evidence-based clinical practice guideline for the use of epoetin in patients with cancer. In cancer patients, the risk of bleeding depends not only on the platelet count, but also on the underlying disease, in accordance with coagulation defects. The cause of thrombocytopenia must be established prior to platelet transfusion since platelet transfusions may be relatively contraindicated in certain conditions e. g., heparin-induced thrombocytopenia(HIT), and thrombotic thrombocytopenic purpura/hemolytic uremic syndrome(TTP/HUS)."

Classic polyarteritis nodosa presenting rare clinical manifestations in a hemophilia A patient Reviewed

"Matsushita, T., Adachi, H., Watanabe, H., Shimoyama, Y., Adachi, T., Sobue, G., Ito, M., Kojima, T., Saito, H. and Naoe, T."

Int. J. Hematol Vol. 83 page： 420-425 2006

Recurrent Intramural Hematoma of the Small Intestine in a Severe Hemophilia A Patient with a High Titer of Factor VIII Inhibitor: A Case Report and Review of the Literature Reviewed

Katsumi, A., Matsushita, T., Hirashima, K., Iwasaki, T., Adachi, T., Yamamoto, K., Kojima, T., Takamatsu, J., Saito, H. and Naoe, T.

Int J Hematol Vol. 84 ( 2 ) page： 166-9 2006

Identification of amino acid residues essential for heparin binding by the A1 domain of human von Willebrand factor. Reviewed

Adachi T, Matsushita T, Dong Z, Katsumi A, Nakayama T, Kojima T, Saito H, Sadler JE, Naoe T.

Biochem Biophys Res Commun Vol. 339 page： 1178-83 2006

In vitro characterization of missense mutations associated with quantitative protein S deficiency Reviewed

Okada, H., Yamazaki, T., Takagi, A., Murate, T., Yamamoto, K., Takamatsu, J., Matsushita, T., Naoe, T., Kunishima, S., Hamaguchi, M., Saito, H. and Kojima, T.

Journal of Thrombosis and Haemostasis Vol. 4 ( 9 ) page： 2003-2009 2006

High titer of ADAMTS13 inhibitor associated with thrombotic microangiopathy of the gut and skeletal muscle after allogenic hematopoietic stem cell transplantation Reviewed

"Adachi, T., Matsushita, T., Ichihashi, R., Hirashima, K., Ito, M., Inukai, A., Yokozawa, T., Nishida, T., Murata, M., Hayashi, M., Katsumi, A., Kojima, T., Saito, H. and Naoe, T."

Int. J. Hematol. Vol. 83 page： 415-419 2006

Severe hemorrhagic complications during remission induction therapy for acute promyelocytic leukemia: incidence, risk factors, and influence on outcome. Reviewed

1. Yanada M, Matsushita T, Asou N, Kishimoto Y, Tsuzuki M, Maeda Y, Horikawa K, Okada M, Ohtake S, Yagasaki F, Matsumoto T, Kimura Y, Shinagawa K, Iwanaga M, Miyazaki Y, Ohno R, Naoe T.

Eur J Haematol Vol. 78 page： 213-219 2007

Molecular basis of antithrombin deficiency in four Japanese patients with antithrombin gene abnormalities including two novel mutations. Reviewed

Kyotani M, Okumura K, Takagi A, Murate T, Yamamoto K, Matsushita T, Sugimura M, Kanayama N, Kobayashi T, Saito H, Kojima T.

Am J Hematol. Vol. 82 ( 8 ) page： 702-705 2007.8

Pitavastatin attenuates the upregulation of tissue factor in restraint-stressed mice. Reviewed

Yamamoto K, Kojima T, Takeshita K, Matsushita T, Takamatsu J.

Thromb Res 2007 Vol. 2007 page： 143-144 2007

An Sp1 binding site mutation of the PROS1 promoter in a patient with protein S deficiency. Reviewed

Sanda N, Fujimori Y, Kashiwagi T, Takagi A, Murate T, Mizutani E, Matsushita T, Naoe T, Kojima T.

Br J Haematol 2007 Vol. 138 page： 663-666 2007

Haematological characteristics of MYH9 disorders due to MYH9 R702 mutations. Reviewed

Kunishima S, Yoshinari M, Nishio H, Ida K, Miura T, Matsushita T, Hamaguchi M, Saito H.

Eur J Haematol. Vol. 78 ( 3 ) page： 220-226 2007

Prognostic analysis of aberrant somatic hypermutation of RhoH gene in diffuse large B cell lymphoma. Reviewed

Hiraga J, Katsumi A, Iwasaki T, Abe A, Kiyoi H, Matsushita T, Kinoshita T, Naoe T.

Leukemia. Vol. 21 ( 8 ) page： 1846-1847 2007

Paratope and epitope mapping of the antithrombotic antibody 6B4 in complex with platelet glycoprotein Ibalpha. Reviewed

Fontayne A, De Maeyer B, De Maeyer M, Yamashita M, Matsushita T, Deckmyn H.

Vol. 282 ( 32 ) page： 23517-23524 2007

A neonate with homozygous protein C deficiency with a homozygous Arg178Trp mutation. Reviewed

Ozlu F, Kyotani M, Taskin E, Ozcan K, Kojima T, Matsushita T, Yapicioğlu H, Takagi A, Saşmaz I, Satar M, Narli N

J Pediatr Hematol Oncol Vol. 30 page： 608-611 2008

A new binding assay of von Willebrand factor and glycoprotein Ib using solid-phase biotinylated platelets.

Hayata K, Nakayama T, Matsushita T, Sakano K

J Pharmacol Sci. Vol. 108 page： 217-221 2008

Mutational analysis of SOS1 gene in acute myeloid leukemia. Reviewed

Tanizaki R, Katsumi A, Kiyoi H, Kunishima S, Iwasaki T, Ishikawa Y, Kobayashi M, Abe A, Matsushita T, Watanabe T, Kojima T, Kaibuchi K, Kojima S, Naoe T.

Int J Hematol. Vol. 88 page： 460-462 2008

Identification and characterization of the first large deletion of the MYH9 gene associated with MYH9 disorders. Reviewed

Kunishima S, Matsushita T, Hamaguchi M, Saito H

Eur J Haematol. Vol. 80 page： 540-544 2008

Identification of amino acid residues responsible for von Willebrand factor binding to sulfatide by charged-to-alanine-scanning mutagenesis. Reviewed

Nakayama T, Matsushita T, Yamamoto K, Mutsuga N, Kojima T, Katsumi A, Nakao N, Sadler JE, Naoe T, Saito H

Int J Hematol Vol. 87 page： 363-370 2008

Prognostic implication and biological roles of RhoH in acute myeloid leukaemia. Reviewed

Iwasaki T, Katsumi A, Kiyoi H, Tanizaki R, Ishikawa Y, Ozeki K, Kobayashi M, Abe A, Matsushita T, Watanabe T, Amano M, Kojima T, Kaibuchi K, Naoe T

Eur J Haematol. Vol. 81 page： 454-460 2008

Ablation of angiotensin IV receptor attenuates hypofibrinolysis via PAI-1 downregulation and reduces occlusive arterial thrombosis. Reviewed

Numaguchi Y, Ishii M, Kubota R, Morita Y, Yamamoto K, Matsushita T, Okumura K, Murohara T

Arterioscler Thromb Vasc Biol. Vol. 29 page： 2102-2108 2009

A novel missense mutation causing abnormal LMAN1 in a Japanese patient with combined deficiency of factor V and factor VIII. Reviewed

Yamada T, Fujimori Y, Suzuki A, Miyawaki Y, Takagi A, Murate T, Sano M, Matsushita T, Saito H, Kojima T

Am J Hematol. Vol. 84 page： 738-742 2009

Definite diagnosis in Japanese patients with protein C deficiency by identification of causative PROC mutations. Reviewed

Takagi A, Tanaka R, Nakashima D, Fujimori Y, Yamada T, Okumura K, Murate T, Yamada M, Horikoshi Y, Yamamoto K, Katsumi A, Matsushita T, Naoe T, Kojima T.

Int J Hematol. Vol. 89 page： 555-557 2009

A novel splice site mutation in intron C of PROS1 leads to markedly reduced mutant mRNA level, absence of thrombin-sensitive region, and impaired secretion and cofactor activity of mutant protein S. Reviewed

Okada H, Kunishima S, Hamaguchi M, Takagi A, Yamamoto K, Takamatsu J, Matsushita T, Saito H, Kojima T, Yamazaki T.

Thromb Res. Vol. 125 page： e246-250 2010

Irrespective of CD34 expression, lineage-committed cell fraction reconstitutes and re-establishes transformed Philadelphia chromosome-positive leukemia in NOD/SCID/IL-2Rgammac-/- mice. Reviewed

Tanizaki R, Nomura Y, Miyata Y, Minami Y, Abe A, Hanamura A, Sawa M, Murata M, Kiyoi H, Matsushita T, Naoe T.

Cancer Sci. Vol. 101 page： 631-638 2010

Heterozygous antithrombin deficiency improves in vivo haemostasis in factor VIII-deficient mice. Reviewed

Bolliger D, Szlam F, Suzuki N, Matsushita T, Tanaka KA

Thromb Haemost. Vol. 103 page： 1233-1238 2010

Severe hemophilia A in a Japanese female caused by an F8-intron 22 inversion associated with skewed X chromosome inactivation. Reviewed

Miyawaki Y, Suzuki A, Fujimori Y, Takagi A, Murate T, Suzuki N, Katsumi A, Naoe T, Yamamoto K, Matsushita T, Takamatsu J, Kojima T.

Int J Hematol. Vol. 92 page： 405-408 2010

Impaired secretion of carboxyl-terminal truncated factor VII due to an F7 nonsense mutation associated with FVII deficiency. Reviewed

Tanaka R, Nakashima D, Suzuki A, Miyawaki Y, Fujimori Y, Yamada T, Takagi A, Murate T, Yamamoto K, Katsumi A, Matsushita T, Naoe T, Kojima T.

Thromb Res. Vol. 125 page： 262-266 2010

Successful fertility management of a patient with factor V deficiency: planned transfusion of fresh frozen plasma under infertility treatment. Reviewed

Iwase A, Goto M, Manabe S, Hirokawa W, Kobayashi H, Nakahara T, Takikawa S, Kotani T, Sumigama S, Tsuda H, Nakayama T, Suzuki N, Matsushita T, Kikkawa F.

Fertil Steril. Vol. 95 page： 2124.e5-7 2011

Pitavastatin-induced angiogenesis and arteriogenesis is mediated by Notch1 in a murine hindlimb ischemia model without induction of VEGF. Reviewed

Kikuchi R, Takeshita K, Uchida Y, Kondo M, Cheng XW, Nakayama T, Yamamoto K, Matsushita T, Liao JK, Murohara T.

Lab Invest. Vol. 91 page： 691-703 2011

Virus-associated hemophagocytic syndrome caused by pandemic swine-origin influenza A (H1N1) in a patient after unrelated bone marrow transplantation. Reviewed

Katsumi A, Nishida T, Murata M, Terakura S, Shimada K, Saito S, Kobayashi M, Kodaira A, Shibata S, Oda I, Yagi T, Kiyoi H, Matsushita T, Kojima T, Naoe T.

J Clin Exp Hematop. Vol. 51 page： 63-65 2011

FLT/ITD regulates leukemia cell adhesion thruough α4β1 integrin ando Pyk2 signalling. Reviewed

Katsumi A, Kiyoi H, Abe A, Tanizaki R, Iwasaki T, Kobayashi M, Matsushita T, Kaibuchi K, Senga T, Kojima T, Kohno T, Hamaguchi M, Naoe T.

Eur J Haematol. Vol. 86 page： 191-198 2011

A novel ENG mutation causing impaired co-translational processing of endoglin associated with hereditary hemorrhagic telangiectasia. Reviewed

Suzuki A, Nakashima D, Miyawaki Y, Fujita J, Maki A, Fujimori Y, Takagi A, Murate T, Teranishi M, Matsushita T, Saito H, Kojima T.

Thromb Res. Vol. 129 page： e200-208 2012

Increased von Willebrand Factor to ADAMTS13 ratio as a predictor of thrombotic complications following a major hepatectomy. Reviewed

Kobayashi S, Yokoyama Y, Matsushita T, Kainuma M, Ebata T, Igami T, Sugawara G, Takahashi Y, Nagino M.

Arch Surg. Vol. 147 page： 909-917 2012

Thrombosis from a prothrombin mutation conveying antithrombin resistance. Reviewed

Miyawaki Y, Suzuki A, Fujita J, Maki A, Okuyama E, Murata M, Takagi A, Murate T, Kunishima S, Sakai M, Okamoto K, Matsushita T, Naoe T, Saito H, Kojima T.

N Engl J Med. Vol. 366 page： 2390-2396 2012

Recombinant factor VIIa analog (vatreptacog alfa [activated]) for treatment of joint bleeds in hemophilia patients with inhibitors: a randomized controlled trial. Reviewed

de Paula EV, Kavakli K, Mahlangu J, Ayob Y, Lentz SR, Morfini M, Nemes L, Šalek SZ, Shima M, Windyga J, Ehrenforth S, Chuansumrit A

J Thromb Haemost. Vol. 10 page： 81-89 2012

Stress augments insulin resistance and prothrombotic state: role of visceral adipose-derived monocyte chemoattractant protein-1. Reviewed

Uchida Y, Takeshita K, Yamamoto K, Kikuchi R, Nakayama T, Nomura M, Cheng XW, Egashira K, Matsushita T, Nakamura H, Murohara T.

Diabetes. Vol. 61 page： 1552-1561 2012

Efficacy and safety of recombinant porcine factor VIII in Japanese patients with acquired hemophilia A

Seki, Y; Ogawa, Y; Kikuchi, T; Sakaida, E; Mizuta, Y; Kitagawa, T; Takemura, K; Miyaguchi, Y; Nogami, K; Matsushita, T

INTERNATIONAL JOURNAL OF HEMATOLOGY Vol. 120 ( 4 ) page： 482 - 491 2024.10

Newly diagnosed multiple myeloma with bleeding and coagulation abnormalities caused by a thrombin-inhibiting substance

Ohara, F; Suzuki, A; Suzuki, N; Kanematsu, T; Okamoto, S; Tamura, S; Kiyoi, H; Matsushita, T; Katsumi, A

INTERNATIONAL JOURNAL OF HEMATOLOGY 2024.9

Variability in combinations of APTT reagent and substrate plasma for a one-stage clotting assay to measure factor VIII products

Suzuki, A; Suzuki, N; Kanematsu, T; Okamoto, S; Suzuki, N; Tamura, S; Kikuchi, R; Katsumi, A; Kojima, T; Matsushita, T

INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY Vol. 46 ( 4 ) page： 705 - 713 2024.8

Fitusiran prophylaxis in people with hemophilia A or B who switched from prior BPA/CFC prophylaxis: the ATLAS-PPX trial

Kenet, G; Nolan, B; Fikar, BZ; Antmen, B; Kampmann, P; Matsushita, T; You, CW; Vilchevska, K; Bagot, CN; Sharif, A; Peyvandi, F; Young, G; Negrier, C; Chi, JR; Kittner, B; Sussebach, C; Shammas, F; Mei, BS; Andersson, S; Kavakli, K

BLOOD Vol. 143 ( 22 ) page： 2256 - 2269 2024.5

Florigen-producing cells express FPF1-LIKE PROTEIN 1 that accelerates flowering and stem growth in long days with sunlight red/far-red ratio in Arabidopsis.

Takagi H, Lee N, Hempton AK, Purushwani S, Notaguchi M, Yamauchi K, Shirai K, Kawakatsu Y, Uehara S, Albers WG, Downing BLR, Ito S, Suzuki T, Matsuura T, Mori IC, Mitsuda N, Kurihara D, Matsushita T, Song YH, Sato Y, Nomoto M, Tada Y, Hanada K, Cuperus JT, Queitsch C, Imaizumi T

bioRxiv : the preprint server for biology 2024.4

Real-world effectiveness and safety of damoctocog alfa pegol in patients with haemophilia A and a history of factor VIII inhibitors: A post-hoc analysis of the ongoing HEM-POWR study

Reding, M; Alvarez-Román, MT; Castaman, G; Janbain, M; Matsushita, T; Meijer, K; Schmidt, K; Oldenburg, J

HAEMOPHILIA Vol. 30 page： 34 - 35 2024.4

Basement membrane extract potentiates the endochondral ossification phenotype of bone marrow-derived mesenchymal stem cell-based cartilage organoids

Notoh, H; Yamasaki, S; Suzuki, N; Suzuki, A; Okamoto, S; Kanematsu, T; Suzuki, N; Katsumi, A; Kojima, T; Matsushita, T; Tamura, S

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS Vol. 701 page： 149583 2024.3

Surgical procedures and haemostatic outcome in patients with haemophilia receiving concizumab prophylaxis during the phase 3 explorer7 and explorer8 trials

Lopez-Jaime, FJ; Barnes, C; Chan, AKC; Linari, S; Matsushita, T; Bovet, J; Odgaard-Jensen, J; Poulsen, LH

HAEMOPHILIA Vol. 30 page： 66 - 67 2024.2

HEM-POWR study interim analysis four: Effectiveness of damoctocog alfa pegol treatment for previously treated patients with haemophilia A

Reding, MT; Román, MTA; Castaman, G; Janbain, M; Matsushita, T; Meijer, K; Schmidt, K; Oldenburg, J

HAEMOPHILIA Vol. 30 page： 105 - 106 2024.2

Interim analyses of the multinational real-world prospective cohort HEM-POWR study evaluating the effectiveness and safety of damoctocog alfa pegol in patients with hemophilia A

Reding, MT; Alvarez-Román, MT; Castaman, G; Janbain, M; Matsushita, T; Meijer, K; Schmidt, K; Oldenburg, J

EUROPEAN JOURNAL OF HAEMATOLOGY Vol. 112 ( 2 ) page： 286 - 295 2024.2

Safety profile of damoctocog alfa pegol: Fourth interim analysis of the real-world HEM-POWR study for previously treated patients with haemophilia A

Reding, MT; Román, MTA; Castaman, G; Janbain, M; Matsushita, T; Meijer, K; Schmidt, K; Oldenburg, J

HAEMOPHILIA Vol. 30 page： 104 - 105 2024.2

[Biology of von Willebrand factor and pathogenesis of von Willebrand disease].

Matsushita T

[Rinsho ketsueki] The Japanese journal of clinical hematology Vol. 65 ( 8 ) page： 756 - 763 2024

Surgical Procedures and Hemostatic Outcome in Patients with Hemophilia Receiving Concizumab Prophylaxis during the Phase 3 explorer7 and explorer8 Trials

Chan, AKC; Barnes, C; Mathias, M; Linari, S; Jaime, FJL; Poulsen, LH; Bovet, J; Odgaard-Jensen, J; Matsushita, T

BLOOD Vol. 142 2023.11

The immunogenicity, safety, and efficacy of N8-GP in previously untreated patients with severe hemophilia A: pathfinder6 end-of-trial results

Kenet, G; Young, G; Chuansumrit, A; Matsushita, T; Yadav, V; Zak, M; Male, C

JOURNAL OF THROMBOSIS AND HAEMOSTASIS Vol. 21 ( 11 ) page： 3109 - 3116 2023.11

Cases of less-than-expected FVIII activity in previously treated patients during post-marketing surveillance of N8-GP

Oldenburg, J; Benson, G; Chowdary, P; Halimeh, S; Matsushita, T; Norland, A; Wahid, MN; Nemes, L

HAEMOPHILIA Vol. 29 ( 6 ) page： 1475 - 1482 2023.11

Haemophilia and cardiovascular disease in Japan: Low incidence rates from ADVANCE Japan baseline data

Nagao, A; Chikasawa, Y; Sawada, A; Kanematsu, T; Yamasaki, N; Takedani, H; Nojima, M; Fujii, T; Suzuki, N; Matsushita, T; Higasa, S; Amano, K; ADVANCE Japan Working Grp

HAEMOPHILIA Vol. 29 ( 6 ) page： 1519 - 1528 2023.11

Use of nonacog beta pegol during surgery in persons with hemophilia B: a case series

Phua, CW; Matino, D; Kühnöl, CD; Hegemann, I; Matsushita, T

RESEARCH AND PRACTICE IN THROMBOSIS AND HAEMOSTASIS Vol. 7 ( 7 ) page： 102208 2023.10

Clinical course and prognosis of patients with hepatocellular carcinoma and haemophilia

Matsuda, N; Imai, N; Yokoyama, S; Yamamoto, K; Ito, T; Ishizu, Y; Honda, T; Okamoto, S; Kanematsu, T; Suzuki, N; Matsushita, T; Ishigami, M; Kawashima, H

EUROPEAN JOURNAL OF GASTROENTEROLOGY & HEPATOLOGY Vol. 35 ( 10 ) page： 1211 - 1215 2023.10

EFFECTIVENESS AND SAFETY OF DAMOCTOCOG ALFA PEGOL IN PATIENTS WITH HEMOPHILIA A WITH A HISTORY OF FACTOR VIII INHIBITORS: INTERIM ANALYSIS FROM THE REAL-WORLD HEM-POWR STUDY

Castaman, G; Reding, MT; Román, MTA; Janbain, M; Peyvandi, F; Matsushita, T; Meijer, K; Schmidt, K; Oldenburg, J

HAEMOPHILIA Vol. 29 page： 32 - 32 2023.9

Phase 3 Trial of Concizumab in Hemophilia with Inhibitors

Matsushita, T; Shapiro, A; Abraham, A; Angchaisuksiri, P; Castaman, G; Cepo, K; d'Oiron, R; Frei-Jones, M; Goh, AS; Haaning, J; Jacobsen, SH; Mahlangu, J; Mathias, M; Nogami, K; Rasmussen, JS; Stasyshyn, O; Tran, H; Vilchevska, K; Martinez, LV; Windyga, J; You, CW; Zozulya, N; Zulfikar, B; Jiménez-Yuste, V

NEW ENGLAND JOURNAL OF MEDICINE Vol. 389 ( 9 ) page： 783 - 794 2023.8

The efficacy and safety of caplacizumab in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura: an open-label phase 2/3 study

Miyakawa, Y; Imada, K; Ichikawa, S; Uchiyama, H; Ueda, Y; Yonezawa, A; Fujitani, S; Ogawa, Y; Matsushita, T; Asakura, H; Nishio, K; Suzuki, K; Hashimoto, Y; Murakami, H; Tahara, S; Tanaka, T; Matsumoto, M

INTERNATIONAL JOURNAL OF HEMATOLOGY Vol. 117 ( 3 ) page： 366 - 377 2023.3

The safety and efficacy of N8-GP (turoctocog alfa pegol) in previously untreated pediatric patients with hemophilia A

Male, C; Königs, C; Dey, S; Matsushita, T; Millner, AH; Zak, M; Young, G; Kenet, G

BLOOD ADVANCES Vol. 7 ( 4 ) page： 620 - 629 2023.2

THIRD INTERIM ANALYSIS OF THE PHASE IV HEM-POWR STUDY EVALUATING THE REAL-WORLD EFFECTIVENESS OF DAMOCTOCOG ALFA PEGOL IN PREVIOUSLY TREATED PATIENTS WITH HEMOPHILIA A

Reding, MT; Roman, MTA; Sanabria, M; Castaman, G; Janbain, M; Matsushita, T; Meijer, K; Schmidt, K; Oldenburg, J

HAEMOPHILIA Vol. 29 page： 116 - 116 2023.2

INTERIM SUBGROUP ANALYSIS OF HEMOPHILIA A SEVERITY ON THE EFFECTIVENESS AND SAFETY OF DAMOCTOCOG ALFA PEGOL FROM THE REAL-WORLD OBSERVATIONAL HEM-POWR STUDY

Reding, M; Roman, MTA; Sanabria, M; Castaman, G; Janbain, M; Matsushita, T; Meijer, K; Schmidt, K; Oldenburg, J

AMERICAN JOURNAL OF HEMATOLOGY Vol. 98 page： E90 - E90 2023.2

The usefulness of tranexamic acid for bleeding symptoms of chronic consumptive coagulopathy complicated by aortic disease: a single-institute, retrospective study of 14 patients

Suzuki, N; Suzuki, N; Kawaguchi, Y; Okamoto, S; Kanematsu, T; Katsumi, A; Suzuki, A; Tamura, S; Kojima, T; Kiyoi, H; Matsushita, T

THROMBOSIS JOURNAL Vol. 21 ( 1 ) page： 10 2023.1

The seroprevalence of neutralizing antibodies against the adeno-associated virus capsids in Japanese hemophiliacs

Kashiwakura, Y; Baatartsogt, N; Yamazaki, S; Nagao, A; Amano, K; Suzuki, N; Matsushita, T; Sawada, A; Higasa, S; Yamasaki, N; Fujii, T; Ogura, T; Takedani, H; Taki, M; Matsumoto, T; Yamanouchi, J; Sakai, M; Nishikawa, M; Yatomi, Y; Yada, K; Nogami, K; Watano, R; Hiramoto, T; Hayakawa, M; Kamoshita, N; Kume, A; Mizukami, H; Ishikawa, S; Sakata, Y; Ohmori, T

MOLECULAR THERAPY-METHODS & CLINICAL DEVELOPMENT Vol. 27 page： 404 - 414 2022.12

The Immunogenicity, Safety and Efficacy of N8-GP in Previously Untreated Patients (PUPs) with Severe Hemophilia A: pathfinder6 End-of-Trial Results

Kenet, G; Chuansumrit, A; Matsushita, T; Young, G; Yadav, V; Zak, M; Male, C

BLOOD Vol. 140 page： 2726 - 2727 2022.11

Efficacy and Safety of Personalized Prophylaxis with Simoctocog Alfa in Adult Japanese Previously Treated Patients with Severe Hemophilia a

Shima, M; Taki, M; Shirayama, R; Matsushita, T; Nishida, Y; Nagao, A; Ogawa, Y; Kawasugi, K

BLOOD Vol. 140 page： 11304 - 11305 2022.11

Fitusiran Prophylaxis Improves Health-Related Quality of Life in People with Hemophilia a or B, with or without Inhibitors: Results of ATLAS-PPX Study

Kenet, G; Nolan, B; Matsushita, T; Young, G; Quan, T; Cano, V; Sussebach, C; Andersson, S; Mei, BS; Afonso, M; Kavakli, K

BLOOD Vol. 140 page： 7977 - 7978 2022.11

Interim Subgroup Analysis of the Effectiveness and Safety of Damoctocog Alfa Pegol Prophylaxis in Previously Treated Patients with Hemophilia A Treated Every 5 or Every 7 Days: Results from the Real-World Observational HEM-POWR Study

Reding, MT; Román, MTA; Sanabria, M; Castaman, G; Janbain, M; Matsushita, T; Meijer, K; Schmidt, K; Oldenburg, J

BLOOD Vol. 140 page： 5612 - 5614 2022.11

Clinical conditions and risk factors for inhibitor-development in patients with haemophilia: A decade-long prospective cohort study in Japan, J-HIS2 (Japan Hemophilia Inhibitor Study 2)

Nogami, K; Taki, M; Matsushita, T; Kojima, T; Oka, T; Ohga, S; Kawakami, K; Sakai, M; Suzuki, T; Higasa, S; Horikoshi, Y; Shinozawa, K; Tamura, S; Yada, K; Imaizumi, M; Ohtsuka, Y; Iwasaki, F; Kobayashi, M; Takamatsu, J; Takedani, H; Nakadate, H; Matsuo, Y; Matsumoto, T; Fujii, T; Fukutake, K; Shirahata, A; Yoshioka, A; Shima, M

HAEMOPHILIA Vol. 28 ( 5 ) page： 745 - 759 2022.9

VWF-Gly2752Ser, a novel non-cysteine substitution variant in the CK domain, exhibits severe secretory impairment by hampering C-terminal dimer formation.

Okamoto S, Tamura S, Sanda N, Odaira K, Hayakawa Y, Mukaide M, Suzuki A, Kanematsu T, Hayakawa F, Katsumi A, Kiyoi H, Kojima T, Matsushita T, Suzuki N

Journal of thrombosis and haemostasis : JTH 2022.5

The change for qualitative balance of vascular endothelial growth factor-a may contribute to therapeutic efficacy for an anti-neutrophil cytoplasmic antibody associated vasculitis subtypes

Kikuchi, R; Naotake, T; Maruyama, S; Murohara, T; Matsushita, T

CLINICA CHIMICA ACTA Vol. 530 page： S205 - S206 2022.5

F9 mRNA splicing aberration due to a deep Intronic structural variation in a patient with moderate hemophilia B.

Odaira K, Kawashima F, Tamura S, Suzuki N, Tokoro M, Hayakawa Y, Suzuki A, Kanematsu T, Okamoto S, Takagi A, Katsumi A, Matsushita T, Shima M, Nogami K, Kojima T, Hayakawa F

Thrombosis research Vol. 213 page： 91 - 96 2022.5

Medical Management of a Mural Thrombus Inducing Repeated Ischemic Strokes in a Patient with Congenital Afibrinogenemia.

Nishihori M, Araki Y, Suzuki N, Tamura S, Hattori M, Izumi T, Goto S, Yokoyama K, Uda K, Matsushita T, Saito R

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association Vol. 31 ( 7 ) page： 106526 2022.4

International consensus recommendations on the management of people with haemophilia B

Hart Daniel P., Matino Davide, Astermark Jan, Dolan Gerard, d'Oiron Roseline, Hermans Cedric, Jimenez-Yuste Victor, Linares Adriana, Matsushita Tadashi, McRae Simon, Ozelo Margareth C., Platton Sean, Stafford Darrel, Sidonio Robert F. Jr., Tiede Andreas

THERAPEUTIC ADVANCES IN HEMATOLOGY Vol. 13 page： 20406207221085202 2022.4

Indoxyl Sulfate Activates NLRP3 Inflammasome to Induce Cardiac Contractile Dysfunction Accompanied by Myocardial Fibrosis and Hypertrophy

Yamaguchi Kazutoshi, Yisireyili Maimaiti, Goto Sumie, Cheng Xian Wu, Nakayama Takayuki, Matsushita Tadashi, Niwa Toshimitsu, Murohara Toyoaki, Takeshita Kyosuke

CARDIOVASCULAR TOXICOLOGY Vol. 22 ( 4 ) page： 365 - 377 2022.4

Long-term efficacy and safety of subcutaneous concizumab prophylaxis in hemophilia A and hemophilia A/B with inhibitors.

Shapiro AD, Angchaisuksiri P, Astermark J, Benson G, Castaman G, Eichler H, Jiménez-Yuste V, Kavakli K, Matsushita T, Poulsen LH, Wheeler AP, Young G, Zupancic-Salek S, Oldenburg J, Chowdary P

Blood advances 2022.3

Periosteum-derived podoplanin-expressing stromal cells regulate nascent vascularization during epiphyseal marrow development.

Tamura S, Mukaide M, Katsuragi Y, Fujii W, Odaira K, Suzuki N, Tsukiji N, Okamoto S, Suzuki A, Kanematsu T, Katsumi A, Takagi A, Ikeda K, Ueyama J, Hirayama M, Suzuki-Inoue K, Matsushita T, Kojima T, Hayakawa F

The Journal of biological chemistry Vol. 298 ( 5 ) page： 101833 2022.3

International Society of Blood Transfusion survey of experiences of blood banks and transfusion services during the COVID-19 pandemic

Al-Riyami Arwa Z., Burnouf Thierry, Wood Erica M., Devine Dana V., Oreh Adaeze, Apelseth Torunn Oveland, Goel Ruchikha, Bloch Evan M., van den Berg Karin, Getshen Mahrukh, Louw Vernon, Ang Ai Leen, Lee Cheuk Kwong, Rahimi-Levene Naomi, Stramer Susan L., Vassallo Ralph, Schulze Torsten J., Patidar Gopal Kumar, Pandey Hem Chandra, Dubey Rounak, Badawi Maha, Hindawi Salwa, Meshi Abdullah, Matsushita Tadashi, Sorrentino Enrico, Grubovic Rastvorceva Rada M., Bazin Renee, Vermeulen Marion, Nahirniak Susan, Tsang Hamilton C., Vrielink Hans, Triyono Teguh, Addas-Carvalho Marcelo, Hecimovic Ana, Torres Oscar W., Mutindu Samclide M., Bengtsson Jesper, Dominguez Diego, Sayedahmed Ahmed, Hanisa Musa Rozi, Gautam Bipul, Herczenik Eszter, So-Osman Cynthia

VOX SANGUINIS 2022.3

AKATSUKI study: a prospective, multicentre, phase IV study evaluating the safety of emicizumab under and immediately after immune tolerance induction therapy in persons with congenital haemophilia A with factor VIII inhibitors

Matsushita Tadashi, Suzuki Nobuaki, Nagao Azusa, Nagae Chiai, Yamaguchi-Suita Haruko, Kyogoku Yui, Ioka Akiko, Nogami Keiji

BMJ OPEN Vol. 12 ( 3 ) page： e057018 2022.3

Assessment of global coagulation function under treatment with emicizumab concomitantly with bypassing agents in haemophilia A with inhibitor (UNEBI Study): multicentre open-label non-randomised clinical trial

Ogiwara Kenichi, Taki Masashi, Suzuki Takashi, Takedani Hideyuki, Matsushita Tadashi, Amano Kagehiro, Matsumoto Masanori, Nishio Kenji, Shima Midori, Kasahara Masato, Nogami Keiji

BMJ OPEN Vol. 12 ( 2 ) page： e056922 2022.2

SURGERIES AND DIAGNOSTIC PROCEDURES IN HAEMOPHILIA PATIENTS ON CONCIZUMAB PROPHYLAXIS IN PHASE 2 CLINICAL TRIALS

Wheeler A., Benson G., Eichler H., Tonder S. M., Cepo K., Jimenez-Yuste V., Kavakli K., Wong L. L., Matsushita T.

HAEMOPHILIA Vol. 28 page： 92 - 92 2022.2

SECOND INTERIM ANALYSIS OF THE PHASE IV HEM-POWR STUDY EVALUATING THE REAL-WORLD EFFECTIVENESS AND SAFETY OF DAMOCTOCOG ALFA PEGOL IN PREVIOUSLY TREATED PATIENTS WITH HAEMOPHILIA A

Reding M. T., Roman M. T. Alvarez, Sanabria M., Castaman G., Janbain M., Matsushita T., Meijer K., Schmidt K., Oldenburg J.

HAEMOPHILIA Vol. 28 page： 89 - 90 2022.2

Protein S-Leu17Pro disrupts the hydrophobicity of its signal peptide causing a proteasome-dependent degradation.

Okada K, Tamura S, Suzuki N, Odaira K, Mukaide M, Fujii W, Katsuragi Y, Suzuki A, Kanematsu T, Okamoto S, Suzuki N, Katsumi A, Matsushita T, Kojima T, Hayakawa F

Thrombosis research Vol. 210 page： 26 - 32 2022.2

DOSE OPTIMISATION AND RISK MITIGATION DURING CONCIZUMAB PROPHYLAXIS IN PATIENTS WITH HAEMOPHILIA A/B WITH AND WITHOUT INHIBITORS IN PHASE 3 CLINICAL TRIALS

Chowdary P., Eichler H., Matsushita T., Rose T. H., Ruzanski C., Seremetis S.

HAEMOPHILIA Vol. 28 page： 27 - 27 2022.2

Development and validation of a novel qualitative test for plasma fibrinogen utilizing clot waveform analysis

Suzuki Atsuo, Suzuki Nobuaki, Kanematsu Takeshi, Shinohara Sho, Kurono Hiroshi, Arai Nobuo, Okamoto Shuichi, Suzuki Naruko, Tamura Shogo, Kikuchi Ryosuke, Katsumi Akira, Kojima Tetsuhito, Matsushita Tadashi

SCIENTIFIC REPORTS Vol. 12 ( 1 ) page： 434 2022.1

International Forum on the Management of Major Haemorrhage: Responses

Green Laura, Stanworth Simon, McQuilten Zoe, Lin Victor, Tucker Harriet, Jackson Bryon, Badawi Maha, Hindawi Salwa, Chaurasia Rahul, Patidar Gopal, Pandey Hem Chandra, Fasola Foluke, Miyata Shigeki, Matsumoto Masanori, Matsushita Tadashi, Rahimi-Levene Naomi, Peer Victoria, Pavenski Katerina, Callum Jeannie, Thompson Troy, Murphy Michael, Staves Julie, Maegele Marc, Abeyakoon Chathuri, Rushford Kylie, Wood Erica, Nunez Maria Antonieta, Mellado Sandra, Saa Edgardo, Triyono Teguh, Pratomo Bhirowo, Apelseth Torunn Oveland, Dunbar Nancy

VOX SANGUINIS 2022.1

International Forum on the Management of Major Haemorrhage: Summary.

Green L, Stanworth S, McQuilten Z, Lin V, Tucker H, Jackson B, Badawi M, Hindawi S, Chaurasia R, Patidar G, Pandey HC, Fasola F, Miyata S, Matsumoto M, Matsushita T, Rahimi-Levene N, Peer V, Pavenski K, Callum J, Thompson T, Murphy M, Staves J, Maegele M, Abeyakoon C, Rushford K, Wood E, Nuñez MA, Mellado S, Saa E, Triyono T, Pratomo B, Apelseth TO, Dunbar N

Vox sanguinis 2022.1

The influence of hepatitis C virus eradication on hepatocarcinogenesis in patients with hemophilia HCC after HCV eradication in hemophilia

Inukai Yosuke, Imai Norihiro, Yamamoto Kenta, Ito Takanori, Ishizu Yoji, Honda Takashi, Okamoto Shuichi, Kanematsu Takeshi, Suzuki Nobuaki, Matsushita Tadashi, Ishigami Masatoshi, Fujishiro Mitsuhiro

ANNALS OF HEPATOLOGY Vol. 27 ( 1 ) page： 100545 2022

Feasibility of the automated column agglutination technique for titration of anti-A/B antibodies in ABO-incompatible living kidney transplantation

Matsuura Hideaki, Sugiura Yukari, Matsuno Takahiro, Tomiya Yume, Shiraki Mari, Kato Chiaki, Ishihara Keiko, Fukami Harue, Niwa Reiko, Hayashi Megumi, Matsushita Tadashi, Kato Hidefumi, Watarai Yoshihiko, Ito Taihei, Kenmochi Takashi, Fujii Sumie, Miura Yasuo

THERAPEUTIC APHERESIS AND DIALYSIS 2021.12

The Efficacy and Safety of Caplacizumab in Japanese Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): An Open-Label, Phase 2/3 Study

Miyakawa, Y; Imada, K; Ichikawa, S; Uchiyama, H; Ueda, Y; Yonezawa, A; Fujitani, S; Handa, H; Matsushita, T; Asakura, H; Nishio, K; Suzuki, K; Hashimoto, Y; Ohshima, S; Tahara, S; Tanaka, T; Matsumoto, M

BLOOD Vol. 138 2021.11

Surgeries and Diagnostic Procedures in Heni ophilia Patients on Concizumab Prophylaxis: Results from the Phase 2 Explorer4 and Explorer5 Trials

Wheeler, AP; Benson, G; Eichler, H; Tonder, SM; Cepo, K; Yuste, VJ; Kavakli, K; Wong, LLL; Matsushita, T

BLOOD Vol. 138 2021.11

A POST-HOC ANALYSIS OF TEMPORARILY DECREASED INCREMENTAL RECOVERY (IR) OBSERVED IN A SUBSET OF PREVIOUSLY UNTREATED PATIENTS (PUPS) WITH HAEMOPHILIA A TREATED WITH N8-GP

Male C., Konigs C., Dey S., Matsushita T., Millner Holm A., Sonnergren H., Young G., Kenet G.

HAEMATOLOGICA Vol. 106 ( 9 ) page： 13 - 13 2021.9

THE SAFETY AND EFFICACY OF N8-GP IN PREVIOUSLY UNTREATED PATIENTS (PUPS) WITH SEVERE HAEMOPHILIA A: INTERIM RESULTS FROM THE MAIN AND EXTENSION PHASES OF PATHFINDER6

Kenet G., Konigs C., Dey S., Matsushita T., Millner Holm A., Sonnergren H., Young G., Male C.

HAEMATOLOGICA Vol. 106 ( 9 ) page： 6 - 7 2021.9

Notch1 haploinsufficiency in mice accelerates adipogenesis

Yamaguchi Kazutoshi, Hayashi Motoharu, Uchida Yasuhiro, Cheng Xian Wu, Nakayama Takayuki, Matsushita Tadashi, Murohara Toyoaki, Takeshita Kyosuke

SCIENTIFIC REPORTS Vol. 11 ( 1 ) page： 16761 2021.8

Anemia in older adults as a geriatric syndrome: A review

Katsumi Akira, Abe Akihiro, Tamura Shogo, Matsushita Tadashi

GERIATRICS & GERONTOLOGY INTERNATIONAL Vol. 21 ( 7 ) page： 549 - 554 2021.7

Concomitant use of bypassing agents with emicizumab for people with haemophilia A and inhibitors undergoing surgery

Jimenez-Yuste Victor, Carlos Rodriguez-Merchan E., Matsushita Tadashi, Andre Holme Pal

HAEMOPHILIA Vol. 27 ( 4 ) page： 519 - 530 2021.7

Clopidogrel response predicts thromboembolic events associated with coil embolization of unruptured intracranial aneurysms: A prospective cohort study

Higashi Eiji, Matsumoto Shoji, Nakahara Ichiro, Hatano Taketo, Ishii Akira, Sadamasa Nobutake, Ohta Tsuyoshi, Ishihara Takuma, Tokunaga Keisuke, Ando Mitsushige, Chihara Hideo, Furuta Konosuke, Hashimoto Tetsuya, Tanaka Koji, Sonoda Kazutaka, Koge Junpei, Takita Wataru, Hashikawa Takuro, Funakoshi Yusuke, Kondo Daisuke, Kamata Takahiko, Tsujimoto Atsushi, Matsushita Takuya, Murai Hiroyuki, Matsuo Keitaro, Kitazono Takanari, Kira Junichi

PLOS ONE Vol. 16 ( 4 ) 2021.4

Essential role of a carboxyl-terminal alpha-helix motif in the secretion of coagulation factor XI

Hayakawa Yuri, Tamura Shogo, Suzuki Nobuaki, Odaira Koya, Tokoro Mahiru, Kawashima Fumika, Hayakawa Fumihiko, Takagi Akira, Katsumi Akira, Suzuki Atsuo, Okamoto Shuichi, Kanematsu Takeshi, Matsushita Tadashi, Kojima Tetsuhito

JOURNAL OF THROMBOSIS AND HAEMOSTASIS Vol. 19 ( 4 ) page： 920 - 930 2021.4

Phase I clinical trial of intra-bone marrow cotransplantation of mesenchymal stem cells in cord blood transplantation

Goto Tatsunori, Murata Makoto, Nishida Tetsuya, Terakura Seitaro, Kamoshita Sonoko, Ishikawa Yuichi, Ushijima Yoko, Adachi Yoshiya, Suzuki Satoshi, Kato Katsuyoshi, Hirakawa Akihiro, Nishiwaki Satoshi, Nishio Nobuhiro, Takahashi Yoshiyuki, Kodera Yoshihisa, Matsushita Tadashi, Kiyoi Hitoshi

STEM CELLS TRANSLATIONAL MEDICINE Vol. 10 ( 4 ) page： 542 - 553 2021.4

Vascular endothelial growth factor (VEGF)-A and VEGF-A(165)b are associated with time to remission of granulomatosis with polyangiitis in a nationwide Japanese prospective cohort study

Kikuchi Ryosuke, Tsuboi Naotake, Sada Ken-Ei, Nakatochi Masahiro, Yokoe Yuki, Suzuki Atsuo, Maruyama Shoichi, Murohara Toyoaki, Matsushita Tadashi, Amano Koichi, Atsumi Tatsuya, Takasaki Yoshinari, Ito Satoshi, Hasegawa Hitoshi, Dobashi Hiroaki, Ito Takafumi, Makino Hirofumi, Matsuo Seiichi

ANNALS OF CLINICAL BIOCHEMISTRY Vol. 58 ( 2 ) page： 86 - 94 2021.3

Impact of variation in reagent combinations for one-stage clotting assay on assay discrepancy in nonsevere haemophilia A

Suzuki Atsuo, Suzuki Nobuaki, Kanematsu Takeshi, Okamoto Shuichi, Tamura Shogo, Kikuchi Ryosuke, Katsumi Akira, Kiyoi Hitoshi, Kojima Tetsuhito, Matsushita Tadashi

INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY Vol. 43 ( 1 ) page： 131 - 138 2021.2

SAFETY AND LONGER-TERM EFFICACY OF CONCIZUMAB PROPHYLAXIS IN PATIENTS WITH HAEMOPHILIA A OR B WITH INHIBITORS: RESULTS FROM THE EXTENSION PART OF THE PHASE 2 EXPLORER4 TRIAL

Shapiro A., Castaman G., Cepo K., Tonder S. M., Matsushita T., Poulsen L. Hvitfeldt, Young G., Zupancic-Salek S., Jimenez Yuste V.

HAEMOPHILIA Vol. 27 page： 128 - 128 2021.2

Myh9 R702C is associated with erythroid abnormality with splenomegaly in mice

Kanematsu Takeshi, Suzuki Nobuaki, Tamura Shogo, Suzuki Atsuo, Ishikawa Yuichi, Katsumi Akira, Kiyoi Hitoshi, Saito Hidehiko, Kunishima Shinji, Kojima Tetsuhito, Matsushita Tadashi

NAGOYA JOURNAL OF MEDICAL SCIENCE Vol. 83 ( 1 ) page： 75 - 86 2021.2

Long-term safety and efficacy of emicizumab for up to 5.8 years and patients' perceptions of symptoms and daily life: A phase 1/2 study in patients with severe haemophilia A

Shima Midori, Nagao Azusa, Taki Masashi, Matsushita Tadashi, Oshida Koichi, Amano Kagehiro, Nagami Sayaka, Okada Norihiro, Maisawa Shingo, Nogami Keiji

HAEMOPHILIA Vol. 27 ( 1 ) page： 81 - 89 2021.1

Safety and efficacy of percutaneous radiofrequency ablation for hepatocellular carcinoma patients with haemophilia

Yamamoto Takafumi, Imai Norihiro, Yamamoto Kenta, Ito Takanori, Ishizu Yoji, Honda Takashi, Okamoto Shuichi, Kanematsu Takeshi, Suzuki Nobuaki, Matsushita Tadashi, Ishigami Masatoshi, Fujishiro Mitsuhiro

HAEMOPHILIA Vol. 27 ( 1 ) page： 100 - 107 2021.1

Design of the HEM-POWR study: a prospective, observational study of real-world treatment with damoctocog alfa pegol in patients with haemophilia A

Sanabria Martin, Alvarez Roman Maria Teresa, Castaman Giancarlo, Janbain Maissaa, Matsushita Tadashi, Meijer Karina, Oldenburg Johannes, Friedl Sabine, Reding M. T.

BMJ OPEN Vol. 11 ( 9 ) page： e044997 2021

[The diagnosis and management of von Willebrand disease].

Matsushita T

[Rinsho ketsueki] The Japanese journal of clinical hematology Vol. 62 ( 8 ) page： 1205 - 1212 2021

[The management strategy for von Willebrand disease].

Matsushita T

[Rinsho ketsueki] The Japanese journal of clinical hematology Vol. 62 ( 5 ) page： 435 - 444 2021

Long-term safety and sustained efficacy for up to 5 years of treatment with recombinant factor IX Fc fusion protein in subjects with haemophilia B: Results from the B-YOND extension study

Pasi K. John, Fischer Kathelijn, Ragni Margaret, Kulkarni Roshni, Ozelo Margareth C., Mahlangu Johnny, Shapiro Amy, P'Ng Stephanie, Chambost Herve, Nolan Beatrice, Bennett Carolyn, Matsushita Tadashi, Winding Bent, Fruebis Joachim, Yuan Huixing, Rudin Dan, Oldenburg Johannes

HAEMOPHILIA Vol. 26 ( 6 ) page： E262 - E271 2020.11

Theory for High-Angular-Resolution Photoelectron Holograms Considering the Inelastic Mean Free Path and the Formation Mechanism of Quasi-Kikuchi Band

Matsushita Tomohiro, Muro Takayuki, Yokoya Takayoshi, Terashima Kensei, Kato Yukako, Matsui Hirosuke, Maejima Naoyuki, Hashimoto Yusuke, Matsui Fumihiko

PHYSICA STATUS SOLIDI B-BASIC SOLID STATE PHYSICS Vol. 257 ( 11 ) 2020.11

Aberrant X chromosomal rearrangement through multi-step template switching during sister chromatid formation in a patient with severe hemophilia A

Tokoro Mahiru, Tamura Shogo, Suzuki Nobuaki, Kakihara Misaki, Hattori Yuna, Odaira Koya, Suzuki Sachiko, Takagi Akira, Katsumi Akira, Hayakawa Fumihiko, Okamoto Shuichi, Suzuki Atsuo, Kanematsu Takeshi, Matsushita Tadashi, Kojima Tetsuhito

MOLECULAR GENETICS & GENOMIC MEDICINE Vol. 8 ( 9 ) page： e1390 2020.9

An overview of the pathfinder clinical trials program: Long-term efficacy and safety of N8-GP in patients with hemophilia A

Matsushita Tadashi, Mangles Sarah

JOURNAL OF THROMBOSIS AND HAEMOSTASIS Vol. 18 page： 26 - 33 2020.9

A Japanese multi-institutional collaborative study of antigen-positive red blood cell (RBC) transfusions in patients with corresponding RBC antibodies

Yamada Chiaki, Takeshita Akihiro, Ohto Hitoshi, Ishimaru Ken, Kawabata Kinuyo, Nomaguchi Yuriko, Haraguchi Yasue, Abe Misao, Sobue Koki, Takenouchi Hiroyuki, Takadate Junko, Kamimura Masami, Katai Akiko, Kasai Daisuke, Minami Yumiko, Sugimoto Tatsuya, Michino Junko, Nagai Kazuhiro, Kumagai Mikako, Hasegawa Yuichi, Ishizuka Keiko, Ohtomo Naoki, Yamada Naotomo, Muroi Kazuo, Matsushita Tadashi, Takahashi Koki

VOX SANGUINIS Vol. 115 ( 5 ) page： 456 - 465 2020.7

A survey of blood transfusion errors in Aichi Prefecture in Japan: Identifying major lapses threatening the safety of transfusion recipients

Ri Masaki, Kasai Masanobu, Kohno Akio, Kondo Masaru, Sawa Masashi, Kinoshita Tomohiro, Sugiura Isamu, Miura Yasuo, Yamamoto Kazuhito, Saito Toshiki I, Ozawa Yukiyasu, Matsushita Tadashi, Kato Hidefumi

TRANSFUSION AND APHERESIS SCIENCE Vol. 59 ( 3 ) page： 102735 2020.6

Higher FVIII:C measured by chromogenic substrate assay than by one-stage assay is associated with silent hemophilic arthropathy

Ogawa Mika, Suzuki Nobuaki, Takahashi Nobunori, Tamura Shogo, Suzuki Atsuo, Suzuki Sachiko, Hattori Yuua, Kakihara Misaki, Kanematsu Takeshi, Kojima Toshihisa, Katsumi Akira, Hayakawa Fumihiko, Kojima Tetsuhito, Ishiguro Naoki, Kiyoi Hitoshi, Matsushita Tadashi

THROMBOSIS RESEARCH Vol. 188 page： 103 - 105 2020.4

An experimental setup for creating and imaging He-4(2)* excimer cluster tracers in superfluid helium-4 via neutron-He-3 absorption reaction

Sonnenschein V, Tsuji Y., Kokuryu S., Kubo W., Suzuki S., Tomita H., Kiyanagi Y., Iguchi T., Matsushita T., Wada N., Kitaguchi M., Shimizu H. M., Hirota K., Shinohara T., Hiroi K., Hayashida H., Guo W., Ito D., Saito Y.

REVIEW OF SCIENTIFIC INSTRUMENTS Vol. 91 ( 3 ) 2020.3

REAL-WORLD EFFECTIVENESS AND SAFETY OF BAY 94-9027 ( DAMOCTOCOG ALFA PEGOL) IN PREVIOUSLY TREATED PATIENTS WITH HAEMOPHILIA A (HEM-POWR): ONLINE PATIENT PORTAL AND LIFE-ACTIVE SUB-STUDY

Oldenburg J., Roman M. T. Alvarez, Castaman G., Janbain M., Matsushita T., Meijer K., Friedl S., Sanabria M., Reding M.

HAEMOPHILIA Vol. 26 page： 100 - 101 2020.2

Safety and efficacy of turoctocog alfa in the prevention and treatment of bleeds in previously untreated paediatric patients with severe haemophilia A: Results from the guardian 4 multinational clinical trial

Yaish Hassan, Matsushita Tadashi, Belhani Meriem, Jimenez-Yuste Victor, Kavakli Kaan, Korsholm Lars, Matytsina Irina, Philipp Claire, Reichwald Kirsten, Wu Runhui

HAEMOPHILIA Vol. 26 ( 1 ) page： 64 - 72 2020.1

Indoxyl Sulfate-induced Vascular Calcification is mediated through Altered Notch Signaling Pathway in Vascular Smooth Muscle Cells

Yamaguchi Kazutoshi, Yisireyili Maimaiti, Goto Sumie, Kato Katsuhiro, Cheng Xian Wu, Nakayama Takayuki, Matsushita Tadashi, Niwa Toshimitsu, Murohara Toyoaki, Takeshita Kyosuke

INTERNATIONAL JOURNAL OF MEDICAL SCIENCES Vol. 17 ( 17 ) page： 2703 - 2717 2020

Subcutaneous concizumab prophylaxis in hemophilia A and hemophilia A/B with inhibitors: phase 2 trial results

Shapiro Amy D., Angchaisuksiri Pantep, Astermark Jan, Benson Gary, Castaman Giancarlo, Chowdary Pratima, Eichler Hermann, Jimenez-Yuste Victor, Kavakli Kaan, Matsushita Tadashi, Poulsen Lone Hvitfeldt, Wheeler Allison P., Young Guy, Zupancic-Salek Silva, Oldenburg Johannes

BLOOD Vol. 134 ( 22 ) page： 1973 - 1982 2019.11

Efficacy and Safety of Subcutaneous Prophylaxis with Concizumab in Patients with Hemophilia a or B with Inhibitors: Results from explorer4, a Phase 2, Randomized, Open-Label, Controlled Trial

Shapiro Amy, Castaman Giancarlo, Cepo Katarina, Poulsen Lone Hvitfeldt, Hollensen Christian, Matsushita Tadashi, Young Guy, Zupancic-Salek Silva, Jimenez-Yuste Victor

BLOOD Vol. 134 2019.11

Phase I Study of Cord Blood Transplantation with Intra-Bone Marrow Injection of Mesenchymal Stem Cells

Goto Tatsunori, Murata Makoto, Nishida Tetsuya, Terakura Seitaro, Kamoshita Sonoko, Ishikawa Yuichi, Ushijima Yoko, Adachi Yoshiya, Kato Katsuyoshi, Hirakawa Akihiro, Nishiwaki Satoshi, Nishio Nobuhiro, Takahashi Yoshiyuki, Kodera Yoshihisa, Matsushita Tadashi, Kiyoi Hitoshi

BLOOD Vol. 134 2019.11

Real-World Effectiveness and Safety of BAY 94-9027 (Damoctocog Alfa Pegol) in Previously Treated Patients with Hemophilia A (HEM-POWR): Online Patient Portal and LIFE-ACTIVE Sub-Study

Oldenburg Johannes, Roman Maria Teresa Alvarez, Castaman Giancarlo, Janbain Maissaa, Matsushita Tadashi, Meijer Karina, Friedl Sabine, Sanabria Martin, Reding Mark

BLOOD Vol. 134 2019.11

Systematic review and analysis of efficacy of recombinant factor IX products for prophylactic treatment of hemophilia B in comparison with rIX-FP

Davis Joanna, Yan Songkai, Matsushita Tadashi, Alberio Lorenzo, Bassett Paul, Santagostino Elena

JOURNAL OF MEDICAL ECONOMICS Vol. 22 ( 10 ) page： 1014 - 1021 2019.10

Performance evaluation of Revohem((TM)) FVIII chromogenic and Revohem((TM)) FIX chromogenic in the CS-5100 autoanalyser

Suzuki Atsuo, Suzuki Nobuaki, Kanematsu Takeshi, Shinohara Sho, Arai Nobuo, Kikuchi Ryosuke, Matsushita Tadashi

INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY Vol. 41 ( 5 ) page： 664 - 670 2019.10

Successful Perioperative Combination of High-Dose FVIII Therapy Followed by Emicizumab in a Patient with Hemophilia A with Inhibitors.

Okamoto S, Suzuki N, Suzuki A, Suzuki S, Tamura S, Suzuki M, Takahashi N, Kojima T, Kanematsu T, Kojima T, Kiyoi H, Ishiguro N, Matsushita T

TH open : companion journal to thrombosis and haemostasis Vol. 3 ( 4 ) page： e364 - e366 2019.10

LIFE-ACTIVE: OBSERVATIONAL STUDY EVALUATING THE PHYSICAL ACTIVITY IN A SUBSET OF DAM OCT OCOG ALFA PEGOL TREATED HEMOPHILIA A PATIENTS WHO ARE ENROLLED IN THE HEM- POWR STUDY

Musi E., Sanabria M., Alvarez Roman M. T., Castaman G., Janbain M., Matsushita T., Meijer K., Oldenburg J., Friedl S., Reding M.

HAEMATOLOGICA Vol. 104 page： 16 - 16 2019.9

Management of Neonatal Alloimmune Thrombocytopenia in Type 1 CD36 Deficiency Pregnant Women

Matsushita Tadashi, Sanda Naomi, Watanabe Tomomi, Endo Hiroko, Kato Chiaki

TRANSFUSION Vol. 59 page： 130A - 130A 2019.9

Apolipoprotein E binds to and reduces serum levels of DNA-mimicking, pyrrolated proteins

Hirose Sayumi, Hioki Yusuke, Miyashita Hiroaki, Hirade Naoya, Yoshitake Jun, Shibata Takahiro, Kikuchi Ryosuke, Matsushita Tadashi, Chikazawa Miho, Itakura Masanori, Zhang Mimin, Nagata Koji, Uchida Koji

JOURNAL OF BIOLOGICAL CHEMISTRY Vol. 294 ( 28 ) page： 11035 - 11045 2019.7

Apparent synonymous mutation F9 c.87A > G causes secretion failure by in-frame mutation with aberrant splicing

Odaira Koya, Tamura Shogo, Suzuki Nobuaki, Kakihara Misaki, Hattori Yuna, Tokoro Mahiru, Suzuki Sachiko, Takagi Akira, Katsumi Akira, Hayakawa Fumihiko, Okamoto Shuichi, Suzuki Atsuo, Kanematsu Takeshi, Matsushita Tadashi, Kojima Tetsuhito

THROMBOSIS RESEARCH Vol. 179 page： 95 - 103 2019.7

Ischaemic events are rare, and the prevalence of hypertension is not high in Japanese adults with haemophilia: First multicentre study in Asia

Nagao Azusa, Suzuki Nobuaki, Takedani Hideyuki, Yamasaki Naoya, Chikasawa Yushi, Sawada Akihiro, Kanematsu Takeshi, Nojima Masanori, Higasa Satoshi, Amano Kagehiro, Fukutake Katsuyuki, Fujii Teruhisa, Matsushita Tadashi, Suzuki Takashi

HAEMOPHILIA Vol. 25 ( 4 ) page： E223 - E230 2019.7

Postmarketing safety and effectiveness of recombinant factor IX (nonacog alfa) in Japanese patients with haemophilia B

Fukutake Katsuyuki, Taki Masashi, Matsushita Tadashi, Sakai Michio, Takata Ami, Yamaguchi Hiromi, Karumori Toshiyuki

HAEMOPHILIA Vol. 25 ( 4 ) page： E247 - E256 2019.7

Molecular basis of SERPINC1 mutations in Japanese patients with antithrombin deficiency

Tamura Shogo, Hashimoto Erika, Suzuki Nobuaki, Kakihara Misaki, Odaira Koya, Hattori Yuna, Tokoro Mahiru, Suzuki Sachiko, Takagi Akira, Katsumi Akira, Hayakawa Fumihiko, Suzuki Atsuo, Okamoto Shuichi, Kanematsu Takeshi, Matsushita Tadashi, Kojima Tetsuhito

THROMBOSIS RESEARCH Vol. 178 page： 159 - 170 2019.6

Factor VIII: Long-established role in haemophilia A and emerging evidence beyond haemostasis

Bannow Bethany Samuelson, Recht Michael, Negrier Claude, Hermans Cedric, Berntorp Erik, Eichler Hermann, Mancuso Maria Elisa, Klamroth Robert, O'Hara Jamie, Santagostino Elena, Matsushita Tadashi, Kessler Craig

BLOOD REVIEWS Vol. 35 page： 43 - 50 2019.5

Inhibitor development, safety and efficacy of Advate((R)) among previously treated patients with hemophilia A in a postmarketing surveillance in Japan

Fukutake Katsuyuki, Taki Masashi, Matsushita Tadashi, Nogami Keiji, Shima Midori, Yoshioka Akira, Takamatsu Junki, Uchikawa Haruhiko, Takagi Hiroshi, Arai Morio, Engl Werner, Shirahata Akira

INTERNATIONAL JOURNAL OF HEMATOLOGY Vol. 109 ( 3 ) page： 336 - 345 2019.3

Clot waveform analysis in Clauss fibrinogen assay contributes to classification of fibrinogen disorders

Suzuki Atsuo, Suzuki Nobuaki, Kanematsu Takeshi, Shinohara Sho, Arai Nobuo, Kikuchi Ryosuke, Matsushita Tadashi

THROMBOSIS RESEARCH Vol. 174 page： 98 - 103 2019.2

Inhibitor development, safety, and efficacy of Advate (R) in previously untreated patients with hemophilia A in a postmarketing surveillance in Japan (vol 109, pg 70, 2019)

Taki Masashi, Fukutake Katsuyuki, Matsushita Tadashi, Nogami Keiji, Shima Midori, Yoshioka Akira, Takamatsu Junki, Arai Morio, Takagi Hiroshi, Uchikawa Haruhiko, Engl Werner, Shirahata Akira

INTERNATIONAL JOURNAL OF HEMATOLOGY Vol. 109 ( 2 ) page： 241 - 241 2019.2

Inhibitor development, safety, and efficacy of Advate((R)) in previously untreated patients with hemophilia A in a postmarketing surveillance in Japan

Taki Masashi, Fukutake Katsuyuki, Matsushita Tadashi, Nogami Keiji, Shima Midori, Yoshioka Akira, Takamatsu Junki, Arai Morio, Takagi Hiroshi, Uchikawa Haruhiko, Engl Werner, Shirahata Akira

INTERNATIONAL JOURNAL OF HEMATOLOGY Vol. 109 ( 1 ) page： 70 - 78 2019.1

B-YOND Final Results Confirm Established Safety, Sustained Efficacy, and Extended Dosing Interval for Up to 4 Years of Treatment With rFIXFc in Previously Treated Subjects With Severe Hemophilia B

Ragni Margaret, Kulkarni Roshni, Pasi K. John, Fischer Kathelijn, Mahlangu Johnny, Shapiro Amy, Nolan Beatrice, Oldenburg Johannes, Matsushita Tadashi, Willemze Annemieke, Yuan Huixing, Rudin Dan

BLOOD Vol. 132 2018.11

Chronic Inflammatory Demyelinating Polyneuropathy With Concurrent Membranous Nephropathy: An Anti-paranode and Podocyte Protein Antibody Study and Literature Survey

Hashimoto Yu, Ogata Hidenori, Yamasaki Ryo, Sasaguri Takakazu, Ko Senri, Yamashita Kenichiro, Xu Zhang, Matsushita Takuya, Tateishi Takahisa, Akiyama Shin'ichi, Maruyama Shoichi, Yamamoto Akifumi, Kira Jun-ichi

FRONTIERS IN NEUROLOGY Vol. 9 2018.11

Once-weekly prophylaxis with 40 IU/kg nonacog beta pegol (N9-GP) achieves trough levels of >15% in patients with haemophilia B: Pooled data from the paradigm (TM) trials

Oldenburg Johannes, Carcao Manuel, Lentz Steven R., Mahlangu Johnny, Mancuso Maria Elisa, Matsushita Tadashi, Negrier Claude, Clausen Wan Hui Ong, Ehrenforth Silke, Young Guy

HAEMOPHILIA Vol. 24 ( 6 ) page： 911 - 920 2018.11

Characterization of lntraoperative Motor Evoked Potential Monitoring for Surgery of the Pediatric Population with Brain Tumors

Motomura Kazuya, Sumita Kayo, Chalise Lushun, Nishikawa Tomohide, Tanahashi Kuniaki, Ohka Fumiharu, Aoki Kosuke, Hirano Masaki, Nakamura Tomohiko, Matsushita Tadashi, Wakabayashi Toshihiko, Natsume Atsushi

WORLD NEUROSURGERY Vol. 119 page： E1052 - E1059 2018.11

Identification of the novel deletion-type PML-RARA mutation associated with the retinoic acid resistance in acute promyelocytic leukemia

Hattori Hikaru, Ishikawa Yuichi, Kawashima Naomi, Akashi Akimi, Yamaguchi Yohei, Harada Yasuhiko, Hirano Daiki, Adachi Yoshiya, Miyao Kotaro, Ushijima Yoko, Terakura Seitaro, Nishida Tetsuya, Matsushita Tadashi, Kiyoi Hitoshi

PLOS ONE Vol. 13 ( 10 ) page： e0204850 2018.10

The Japanese Immune Tolerance Induction (J-ITI) study in haemophilia patients with inhibitor: Outcomes and successful predictors of ITI treatment

Nogami K., Taki M., Matsushita T., Ohga S., Oka T., Horikoshi Y., Amano K., Shima M.

HAEMOPHILIA Vol. 24 ( 5 ) page： E328 - E337 2018.9

Perioperative safety and hemostatic efficacy of Advate(A (R)) in patients with hemophilia A in a postmarketing surveillance in Japan

Nogami Keiji, Takedani Hideyuki, Shima Midori, Yoshioka Akira, Matsushita Tadashi, Takamatsu Junki, Taki Masashi, Fukutake Katsuyuki, Uchikawa Haruhiko, Takagi Hiroshi, Arai Morio, Engl Werner, Shirahata Akira

INTERNATIONAL JOURNAL OF HEMATOLOGY Vol. 108 ( 1 ) page： 22 - 29 2018.7

Changes in bleeding and daily life with emicizumab prophylaxis: a questionnaire in patients with hemophilia A with inhibitors (PwHAwI) and their families in a long-term phase 1/2 study

Suzuki Takashi, Taki Masashi, Matsushita Tadashi, Sato Tetsuji, Fukutake Katsuyuki, Kasai Ryu, Okada Norihiro, Maisawa Shingo, Nogami Keiji, Shima Midori

HAEMOPHILIA Vol. 24 page： 32-32 2018.5

Surgical Experience in Two Multicentre, Open-label Phase 3 Studies of Emicizumab in Persons with Haemophilia A with Inhibitors (HAVEN 1 and HAVEN 2)

Kruse-Jarres R., Callaghan M., Croteau S. E., Jimenez-Yuste V., Khoo L., Liesner R., Matsushita T., Recht M., Young G., Chang T., Dhalluin C., Mu Y., Xu J., Devenport J., Ko R., Solari P., Oldenburg J.

BRITISH JOURNAL OF HAEMATOLOGY Vol. 181 page： 128-128 2018.4

Angiotensin receptor blocker irbesartan reduces stress-induced intestinal inflammation via AT1a signaling and ACE2-dependent mechanism in mice.

Yisireyili M, Uchida Y, Yamamoto K, Nakayama T, Cheng XW, Matsushita T, Nakamura S, Murohara T, Takeshita K

Brain, behavior, and immunity Vol. 69 page： 167 - 179 2018.3

Vwf K1362A resulted in failure of protein synthesis in mice.

Sanda N, Suzuki N, Suzuki A, Kanematsu T, Kishimoto M, Hasuwa H, Takagi A, Kojima T, Matsushita T, Nakamura S

International journal of hematology 2018.2

Guardian (TM) 4: safety and efficacy of turoctocog alfa in prevention and treatment of bleeds in previously untreated patients with hemophilia A

Ozelo M., Wu R., Belhani M., Lebedev V., Yaish H., Korsholm L., Matytsina I., Matsushita T.

HAEMOPHILIA Vol. 24 page： 101-102 2018.2

Structural and functional insights into S-thiolation of human serum albumins

Nakashima Fumie, Shibata Takahiro, Kamiya Kohei, Yoshitake Jun, Kikuchi Ryosuke, Matsushita Tadashi, Ishii Isao, Gimenez-Bastida Juan A., Schneider Claus, Uchida Koji

SCIENTIFIC REPORTS Vol. 8 ( 1 ) page： 932 2018.1

Combined deficiency of factors V and VIII by chance coinheritance of parahaemophilia and haemophilia A, but not by mutations of either LMAN1 or MCFD2, in a Japanese family

Suzuki S., Nakamura Y., Suzuki N., Yamazaki T., Takagi Y., Tamura S., Takagi A., Kanematsu T., Matsushita T., Kojima T.

HAEMOPHILIA Vol. 24 ( 1 ) page： e13-e16 2018.1

Efficacy and safety of full-length pegylated recombinant factor VIII with extended half-life in previously treated patients with hemophilia A: comparison of data between the general and Japanese study populations (vol 106, pg 704, 2017)

Nogami Keiji, Shima Midori, Fukutake Katsuyuki, Fujii Teruhisa, Taki Masashi, Matsushita Tadashi, Higasa Satoshi, Sato Tetsuji, Sakai Michio, Arai Morio, Uchikawa Haruhiko, Engl Werner, Abbuehl Brigitt, Konkle Barbara A.

INTERNATIONAL JOURNAL OF HEMATOLOGY Vol. 107 ( 1 ) page： 123 - 124 2018.1

Pyrometallurgical Separation of Indium Phosphide through the Phosphorous Removal by Iron and the Chlorination Process Utilizing Ammonium Chloride

Terakado Osamu, Matsushita Taishi, Tani Haruki, Hirasawa Masahiro

MATERIALS TRANSACTIONS Vol. 59 ( 6 ) page： 984 - 988 2018

Urinary and circulating levels of the anti-angiogenic isoform of vascular endothelial growth factor-A in patients with chronic kidney disease

Kikuchi Ryosuke, Yasuda Yoshinari, Nakatochi Masahiro, Shibata Yohei, Hara Toshiaki, Suzuki Atsuo, Imaizumi Takahiro, Suzuki Susumu, Ishii Hideki, Takeshita Kyosuke, Matsushita Tadashi, Maruyama Shoichi, Murohara Toyoaki

CLINICA CHIMICA ACTA Vol. 475 page： 102 - 108 2017.12

Efficacy of Early Fusion With Local Bone Graft and Platelet-Rich Plasma in Lumbar Spinal Fusion Surgery Followed Over 10 Years.

Imagama S, Ando K, Kobayashi K, Ishikawa Y, Nakamura H, Hida T, Ito K, Tsushima M, Matsumoto A, Morozumi M, Tanaka S, Machino M, Ota K, Nakashima H, Takamatsu J, Matsushita T, Nishida Y, Ishiguro N, Matsuyama Y

Global spine journal Vol. 7 ( 8 ) page： 749 - 755 2017.12

INTERNATIONAL JOURNAL OF HEMATOLOGY Vol. 106 ( 5 ) page： 704 - 710 2017.11

Long-term safety and efficacy of emicizumab in a phase 1/2 study in patients with hemophilia A with or without inhibitors

Shima Midori, Hanabusa Hideji, Taki Masashi, Matsushita Tadashi, Sato Tetsuji, Fukutake Katsuyuki, Kasai Ryu, Yoneyama Koichiro, Yoshida Hiroki, Nogami Keiji

BLOOD ADVANCES Vol. 1 ( 22 ) page： 1891 - 1899 2017.10

Successful Blood Transfusion Management of a Living Donor Liver Transplant Recipient in the Presence of Anti-Jr(a): A Case Report

Kurata N., Onishi Y., Kamei H., Hori T., Komagome M., Kato C., Matsushita T., Ogura Y.

TRANSPLANTATION PROCEEDINGS Vol. 49 ( 7 ) page： 1604-1607 2017.9

Phase II study of intrabone single unit cord blood transplantation for hematological malignancies

Murata Makoto, Maeda Yoshinobu, Masuko Masayoshi, Onishi Yasushi, Endo Tomoyuki, Terakura Seitaro, Ishikawa Yuichi, Iriyama Chisako, Ushijima Yoko, Goto Tatsunori, Fujii Nobuharu, Tanimoto Mitsune, Kobayashi Hironori, Shibasaki Yasuhiko, Fukuhara Noriko, Inamoto Yoshihiro, Suzuki Ritsuro, Kodera Yoshihisa, Matsushita Tadashi, Kiyoi Hitoshi, Naoe Tomoki, Nishida Tetsuya

CANCER SCIENCE Vol. 108 ( 8 ) page： 1634 - 1639 2017.8

Pharmacokinetics of a novel extended half-life glycoPEGylated factor IX, nonacog beta pegol (N9-GP) in previously treated patients with haemophilia B: results from two phase 3 clinical trials

Tiede A., Abdul-Karim F., Carcao M., Persson P., Clausen W. H. O., Kearney S., Matsushita T., Negrier C., Oldenburg J., Santagostino E., Young G.

HAEMOPHILIA Vol. 23 ( 4 ) page： 547-555 2017.7

Adductome-based identification of biomarkers for lipid peroxidation

Shibata Takahiro, Shimizu Kazuma, Hirano Keita, Nakashima Fumie, Kikuchi Ryosuke, Matsushita Tadashi, Uchida Koji

JOURNAL OF BIOLOGICAL CHEMISTRY Vol. 292 ( 20 ) page： 8223 - 8235 2017.5

Xanthine oxidase inhibition by febuxostat attenuates stress-induced hyperuricemia, glucose dysmetabolism, and prothrombotic state in mice

Yisireyili Maimaiti, Hayashi Motoharu, Wu Hongxian, Uchida Yasuhiro, Yamamoto Koji, Kikuchi Ryosuke, Hamrah Mohammad Shoaib, Nakayama Takayuki, Cheng Xian Wu, Matsushita Tadashi, Nakamura Shigeo, Niwa Toshimitsu, Murohara Toyoaki, Takeshita Kyosuke

SCIENTIFIC REPORTS Vol. 7 ( 1 ) page： 1266 2017.4

Long-term Efficacy of Recombinant Factor IX Fc Fusion Protein (rFIXFc) Prophylaxis in Adults, Adolescents, and Children With Hemophilia B: Longitudinal Analysis of B-LONG, Kids B-LONG, and B-YOND

Kulkarni R., Ragni M., Mahlangu J., Shapiro A., Oldenburg J., Matsushita T., Nolan B., Feng J., Winding B., Ramirez-Santiago A., Barnowski C.

HAEMOPHILIA Vol. 23 page： 23-24 2017.4

Long-term safety and efficacy of extended-interval prophylaxis with recombinant factor IX Fc fusion protein (rFIXFc) in subjects with haemophilia B

Pasi K. John, Fischer Kathelijn, Ragni Margaret, Nolan Beatrice, Perry David J., Kulkarni Roshni, Ozelo Margareth, Mahlangu Johnny, Shapiro Amy D., Baker Ross I., Bennett Carolyn M., Barnes Christopher, Oldenburg Johannes, Matsushita Tadashi, Yuan Huixing, Ramirez-Santiago Alejandra, Pierce Glenn F., Allen Geoffrey, Mei Baisong

THROMBOSIS AND HAEMOSTASIS Vol. 117 ( 3 ) page： 508-518 2017.3

Retrospective analysis of in vivo recovery and clearance during continuous infusion of recombinant factor VIII products: a single-institution study

Suzuki N., Hirakawa A., Kishimoto M., Kanematsu T., Ogawa M., Kiyoi H., Matsushita T.

HAEMOPHILIA Vol. 23 ( 2 ) page： 215-221 2017.3

Individualised Prophylaxis with Recombinant Factor IX FC Fusion Protein (RFIXFC) in Adults/Adolescents With Haemophilia B: Updated Interim Results of the B-YOND Extension Study

Mahlangu J. N., Shapiro A. D., Pasi K. J., Ragni M. V., Ozelo M. C., Oldenburg J., Matsushita T., Baker R. I., Yuan H., Ferrante F., Ramirez-Santiago A., Lethagen S., Barnowski C.

HAEMOPHILIA Vol. 23 page： 85-86 2017.2

A phase III clinical trial of a mixture agent of plasma-derived factor VIIa and factor X (MC710) in haemophilia patients with inhibitors

Shinkoda Y., Shirahata A., Fukutake K., Takamatsu J., Shima M., Hanabusa H., Mugishima H., Takedani H., Kawasugi K., Taki M., Matsushita T., Tawa A., Nogami K., Higasa S., Kosaka Y., Fujii T., Sakai M., Migita M., Uchiba M., Kawakami K., Sameshima K., Ohashi Y., Saito H.

HAEMOPHILIA Vol. 23 ( 1 ) page： 59-66 2017.1

[Biomarkers of Leukemia ~Description of the Practical Use and Operation in the Clinical Laboratory~]-.

Kajiura Y, Yamamoto Y, Okada K, Hattori H, Matsumoto H, Kishimoto M, Matsushita T

Rinsho byori. The Japanese journal of clinical pathology Vol. 65 ( 1 ) page： 93 - 99 2017.1

Acquired immune-mediated von Willebrand syndrome accompanied by antiphospholipid syndrome.

Kobayashi N, Ogawa Y, Yanagisawa K, Ishizaki T, Uchiumi H, Suzuki N, Matsushita T, Ichinose A, Handa H

[Rinsho ketsueki] The Japanese journal of clinical hematology Vol. 58 ( 6 ) page： 613-618 2017

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Papers - MATSUSHITA, Tadashi