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| Visiting Scientist |
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Satoshi Ishii |
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| Research project | ： | Study of the pathogenic mechanism and the search for therapeutic agents in a mouse model for Fabry disease
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| |  | | | | 1) | A symptomatic Fabry disease mouse model generated by
inducing globotrialceramide synthesis. Taguchi
T, Maruyama H, Nameta M, Yamamoto T, Matsuda J, Kulkarni A, Yoshioka H,
and Ishii S Biochem J 456, 373-383, 2013. |  | | | | | 2) | Screening
of Male Dialysis Patients for Fabry Disease by Plasma
Globotriaosylsphingosine. Maruyama H, Takata T, Tsubata Y, Tazawa
R, Goto K, Tohyama J, Narita I, Yoshioka H, Ishii
S. Clin J Am Soc Nephrol. 2013 :629-636. | | | | | | 3) | Pharmacological chaperone therapy for Fabry disease. Ishii S. Proc Jpn Acad Ser B 88. 2012 :18-30. | | | | | | 4) | Preclinical
efficacy and safety of 1-deoxygalactonojirimycin in mice for Fabry
disease. Ishii S, Chang HH, Yoshioka H, Shimada T, Mannen K, Higuchi Y,
Taguchi A, Fan JQ. J Pharmacol Exp Ther. 2009 Mar;328(3):723-31. | | | | | | 5) | Rescue
of mutant a-galactosidase A in the endoplasmic reticulum by
1-deoxygalactonojirimycin leads to trafficking to lysosomes. Hamanaka
R, Shinohara T, Yano S, Nakamura M, Yasuda A, Yokoyama S, Fan JQ,
Kawasaki K, Watanabe M, Ishii S. Biochim Biophys Acta. 2008
Jun;1782(6):408-13. | | | | | | 6) | Naked
plasmid DNA-based a-galactosidase A gene transfer partially reduces
systemic accumulation of globotriaosylceramide in Fabry mice. Nakamura
G, Maruyama H, Ishii S, Shimotori M, Kameda S, Kono T, Miyazaki J,
Kulkarni AB, Gejyo F. Mol Biotechnol. 2008 Feb;38(2):109-19. | | | | | | 7) | Novel
mutations of the GLA gene in Japanese patients with Fabry disease and
their functional characterization by active site specific chaperone.
Shimotori M, Maruyama H, Nakamura G, Suyama T, Sakamoto F, Itoh M,
Miyabayashi S, Ohnishi T, Sakai N, Wataya-Kaneda M, Kubota M, Takahashi
T, Mori T, Tamura K, Kageyama S, Shio N, Maeba T, Yahagi H, Tanaka M,
Oka M, Sugiyama H, Sugawara T, Mori N, Tsukamoto H, Tamagaki K, Tanda
S, Suzuki Y, Shinonaga C, Miyazaki J, Ishii S, Gejyo F. Hum Mutat. 2008
Feb;29(2):331. | | | | | | 8) | Mutant
a-galactosidase A enzymes identified in Fabry disease patients with
residual enzyme activity: biochemical characterization and restoration
of normal intracellular processing by 1-deoxygalactonojirimycin. Ishii
S, Chang HH, Kawasaki K, Yasuda K, Wu HL, Garman SC, Fan JQ. Biochem J.
2007 Sep 1;406(2):285-95. | |
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