研究者詳細 - 西口　康二

論文 - 西口　康二

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Defects in RGS9 or its anchor protein R9AP in patients with slow photoreceptor deactivation. 査読有り

Nature 427 巻 ( 6969 ) 頁： 75-78 2004年

Novel mutations in the cellular retinaldehyde-binding protein gene (RLBP1) associated with retinitis punctata albescens: evidence of interfamilial genetic heterogeneity and fundus changes in heterozygotes 査読有り

Fishman GA, Roberts MF, Derlacki DJ, Grimsby JL, Yamamoto H, Sharon D, Nishiguchi KM, Dryja TP

Arch Ophthalmol 122 巻 ( 1 ) 頁： 70-75 2004年

Mutation screening of the phosducin gene PDC in patients with retinitis pigmentosa and allied diseases 査読有り

Mol Vis 10 巻頁： 62-64 2004年

A novel mutation (I143NT) in guanylate cyclase-activating protein 1 (GCAP1) associated with autosomal dominant cone degeneration 査読有り

45 巻頁： 3863-3870 2004年

Recessive NRL mutations in patients with clumped pigmentary retinal degeneration and relative preservation of blue cone function 査読有り

Nishiguchi KM, Friedman JS, Sandberg MA, Swaroop A, Berson EL, Dryja TP

101 巻頁： 17819-17824 2004年

Cone cGMP-gated channel mutations and clinical findings in patients with achromatopsia, macular degeneration, and other hereditary cone diseases 査読有り

25 巻頁： 248-258 2005年

Recessive mutations in the CYP4V2 gene in East Asian and Middle Eastern patients with Bietti crystalline corneoretinal dystrophy 査読有り

Lin J, Nishiguchi KM, Nakamura M, Dryja TP, Berson EL, Miyake Y

J Med Genet 42 巻 2005年

Retinopathy mutations in the bZIP protein NRL alter phosphorylation and transcriptional activity 査読有り

Hum Mutat 28 巻頁： 589-598 2007年

*The role of VEGF and VEGFR2/Flk1 in proliferation of retinal progenitor cells in murine retinal degeneration 査読有り

48 巻頁： 4315-4320 2007年

*Identification of photoreceptor precursors in the pars plana during ocular development and after retinal injury 査読有り

Nishiguchi KM, Nakamura M, Kaneko H, Kachi S, Terasaki H

Invest Ophthalmol Vis Sci 49 巻頁： 422-428 2008年1月

*Characteristics of Bone Marrow-Derived Microglia in the Normal and Injured Retina 査読有り

Kaneko H, Nishiguchi KM, Nakamura M, Kachi S, Terasaki H

49 巻頁： 4162-4168 2008年9月

*Retardation of Photoreceptor Degeneration in the Detached Retina of rd1 Mouse 査読有り

Kaneko H, Nishiguchi KM, Nakamura M, Kachi S, Terasaki H

Invest Ophthalmol Vis Sci 49 巻頁： 781-787 2008年2月

TRPM1 mutations are associated with the complete form of congenital stationary night blindness 査読有り

Nakamura M, Sanuki R, Yasuma TR, Onishi A, Nishiguchi KM, Koike C, Kadowaki M, Kondo M, Miyake Y, Furukawa T.

Mol Vis 16 巻頁： 425-437 2010年

Regulation of pathologic retinal angiogenesis and inhibition of VEGF-VEGFR2 binding by soluble heparan sulfate. 査読有り

Nishiguchi KM, Kataoka K, Kachi S, Komeima K, Terasaki H

PLoS ONE 5 巻頁： e13493 2010年

The roles of vitreal macrophages and circulating leukocytes in retinal neovascularization. 査読有り

Kataoka K, Nishiguchi KM, Kaneko H, van Rooijen N, Kachi S, Terasaki H

Invest Ophthalmol Vis Sci in press 巻 2010年

EVALUATION OF RETINAL ARTERIOLES IN RETINITIS PIGMENTOSA: Arterial Lumen Diameter Reduced With Retinal Degeneration and Wall Thickness Related to Systemic Condition.

Ota J, Inooka T, Tomita R, Kominami T, Koyanagi Y, Ito Y, Terasaki H, Nishiguchi KM, Ueno S

Retina (Philadelphia, Pa.) 45 巻 ( 3 ) 頁： 532 - 540 2025年3月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：Retina

Purpose:Retinal arterioles are attenuated in eyes with retinitis pigmentosa, but dimensions such as lumen diameter and wall thickness have not been thoroughly investigated. The aim of the study was to determine the dimensions of reduced retinal arterioles in eyes with retinitis pigmentosa.Methods:The study included 66 patients with retinitis pigmentosa (66 eyes) and 36 control participants (36 eyes) for whom adaptive optics recordings of the retinal arteries were available. Lumen diameter and wall thickness were measured on the adaptive optics images. The correlations between arteriole dimensions and clinical parameters were determined.Results:The mean lumen diameter was significantly smaller in patients with retinitis pigmentosa than in controls, while the mean wall thickness did not differ significantly. Lumen diameter was significantly and positively correlated with the mean deviation of the Humphrey 10-2 field analyzer and ellipsoid zone width. The wall thickness and wall-to-lumen ratio were significantly and positively correlated with age and systolic blood pressure but not with mean deviation and ellipsoid zone width.Conclusion:Retinal arteriole dimensions were significantly correlated with retinal degeneration and systemic factors.

DOI： 10.1097/IAE.0000000000004333

Scopus

PubMed

Plate Manufacturing Constraint in Topology Optimization Using Anisotropic Filter

Wada, Y; Shimada, T; Nishiguchi, K; Okazawa, S; Tsubokura, M

COMPUTER-AIDED DESIGN 180 巻 2025年3月

出版者・発行元：CAD Computer Aided Design

The manufacturing constraint of topology optimization for finding structures composed primarily of plate cross sections with an automatically decided surface normal is discussed. The anisotropic partial differential equation filter for the sensitivity of the objective function is designed such that the eigenvalue-resolved stress tensor is converted to the anisotropic filter tensor. The user can obtain an optimal shape proposal with different degrees of plate formation by changing the influence radius of the ellipsoidal filter. Plate structure formation and stiffness compliance performance are discussed through three sample problems: a solid bar subjected to torsional loads, an L-shaped member subjected to multiple loads, and a vehicle body frame intended to be a complex frame. The finite element analysis with 3–300 million degrees of freedom is required to form a shell plate structure from a large ingot with a volume fraction constraint of 1%–5%. A voxel topology optimization software that utilizes the building cube method framework available in massively parallel environments is developed, and the parallel performance of the optimization routine, including the plate filtering, is measured.

DOI： 10.1016/j.cad.2024.103823

Web of Science

Scopus

Silicone oil, an intraocular surgical adjuvant, induces retinal ferroptosis

Shimizu, H; Tanaka, H; Tazaki, A; Yamada, K; Suzumura, A; Ota, J; Ushio-Watanabe, N; Zheng, H; Kataoka, K; Hara, H; Nishikawa, Y; Yasukawa, T; Suzuma, K; Terasaki, H; Nishiguchi, KM; Kato, M; Toyokuni, S; Kaneko, H

FREE RADICAL BIOLOGY AND MEDICINE 228 巻頁： 33 - 43 2025年2月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：Free Radical Biology and Medicine

Vitrectomy with silicone oil (SO) endotamponade is an effective treatment for vision-threatening retinal diseases. However, unexplained vision impairment has been reportedly critical side effects. Previously, we reported that the eyes with ocular toxoplasmosis showed retinal ferroptosis with the clinical sign of reduced intravitreal iron (Fe). We also found that total iron levels in sub-silicone oil fluid (SOF) in eyes with SO endotamponade were significantly reduced. We hypothesized that the cause of complications related to SO endotamponade is retinal ferroptosis and that low total iron in SOF is a secondary change that occurs similarly to the changes in ocular toxoplasmosis. In this study, we measured total iron levels in ocular fluid from patients, rabbits with SO endotamponade. Retinal iron taken up from the SOF was evaluated using laser ablation inductively coupled plasma mass spectrometry in human and rabbit eyes. Retinal ferroptosis was confirmed by immunohistochemistry of 4-hydrox-2-nonenal-modified proteins, FeRhoNox-1 staining, western blotting and RT-PCR. We found low total iron levels in the SOF, increased oxidative stress and Fe uptake from the SOF into the retinae of human and rabbit eyes, as well as decreased GPx4 expression, increased FeRhoNox-1 signals and altered Fe-related gene expression in SO-filled rabbit eyes. Of note, the target of ferroptosis was Müller cells. We generated an in vitro silicone oil-filled eye model using MIO-M1 cells (a human Müller cell line). The in vitro SO-filled eye model showed decreased GPx4 expression and increased intracellular catalytic Fe(II), an increase in ferroptosis, prevention of cell death by ferrostatin-1, a ferroptosis inhibitor, and altered Fe-related gene expression. These results indicate that the cause of complications related to SO endotamponade was the induction of retinal (Müller cell) ferroptosis, which can be prevented by ferrostatin-1.

DOI： 10.1016/j.freeradbiomed.2024.12.039

Web of Science

Scopus

PubMed

Classification of fundus autofluorescence images based on macular function in retinitis pigmentosa using convolutional neural networks

Kominami, T; Ueno, S; Ota, J; Inooka, T; Oda, M; Mori, K; Nishiguchi, KM

JAPANESE JOURNAL OF OPHTHALMOLOGY 2025年2月

Phenotypic Distinctions Between EYS- and USH2A-Associated Retinitis Pigmentosa in an Asian Population.

Yeo EYH, Kominami T, Tan TE, Babu L, Ong KGS, Tan W, Bylstra YM, Jain K, Tang RWC, Farooqui SZ, Kam SPR, Chan CM, Mathur RS, Jamuar SS, Lim WK, Nishiguchi K, Fenner BJ

Translational vision science & technology 14 巻 ( 2 ) 頁： 16 2025年2月

記述言語：英語出版者・発行元：Translational vision science & technology

Purpose: This study compares clinical characteristics of retinitis pigmentosa (RP) associated with mutations in the EYS and USH2A genes in a Southeast Asian cohort. Methods: Prospective single-center study of families with EYS- or USH2A-associated RP seen at the Singapore National Eye Centre. Comprehensive ophthalmic evaluations, multimodal imaging, genetic testing, and longitudinal follow-up identified clinically useful differentiating features between the two genotypes. Results: A total of 300 families with RP were enrolled, with EYS- and USH2A-associated RP, accounting for 24.7% of all probands and 50.7% of solved or likely solved cases. USH2A cases were predominantly nonsyndromic RP (75%). EYS-associated RP was more severe in functional and structural outcomes, and patients were more myopic than USH2A (SE -3.31 vs. -0.69; P < 0.0001). EYS RP displayed peripapillary nasal sparing on autofluorescence imaging more frequently than USH2A (57.6% vs. 26.7%; P = 0.006), whereas USH2A cases more often had a parafoveal ring (73.3% vs. 30.3%; P = 0.0002). Multiple logistic regression identified diagnostic features with 83.2% accuracy in distinguishing between EYS and USH2A, validated in a second unrelated clinical cohort. Conclusions: EYS- and USH2A-associated RP have overlapping clinical presentations but can often be distinguished based on a constellation of phenotypic features including disease onset and severity, refractive error, and fundus autofluorescence. These diagnostic features may support a more effective diagnostic strategy for these common forms of RP. Translational Relevance: Distinct clinical features differentiating EYS- and USH2A-associated RP provide valuable diagnostic tools that may inform personalized management and facilitate targeted interventions in clinical practice.

DOI： 10.1167/tvst.14.2.16

Scopus

PubMed

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