石井達 (Satoshi Ishii) - マイポータル

基本情報

所属: 大分大学医学部マトリックス医学講座客員研究員
: 株式会社グライコファーマ代表取締役

学位: 博士（薬学）(東京薬科大学)
修士（薬学）(東京薬科大学)

J-GLOBAL ID: 200901024177051980
researchmap会員ID: 5000067163

研究キーワード

研究分野

経歴

2009年 - 現在

大分大学医学部, マトリックス医学講座, 客員研究員
2003年 - 現在

Professor, Obihiro University of
2003年 - 2009年

帯広畜産大学, 畜産学部, 教授
2001年 - 2002年

米国マウントサイナイ医科大学客員教授
2001年 - 2002年

Visiting Associate Professor, Mount Sinai
1996年 - 2002年

うすき生物科学研究所主席研究員
1996年 - 2002年

Cheif Research Scientist, Usuki Bio
1990年 - 1995年

（財）東京都臨床医学総合研究所研究員
1990年 - 1995年

Researcher, The Tokyo Metropolitan
1986年 - 1989年

米国ワシントン大学医学部ポスドク研究員
1986年 - 1989年

Post-Doctral Fellow, School of Medicine,
Agriculture and Veterinary Medicine
School of Medicine
Research Center
Institute of Medical Science
Washington University

学歴

受賞

1995年

上原記念生命科学財団研究員奨励賞
1995年

Research Award from the Uehara Memorial Foundation

論文

Correction: Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis.

Hiroki Maruyama, Kaori Miyata, Mariko Mikame, Atsumi Taguchi, Chu Guili, Masaru Shimura, Kei Murayama, Takeshi Inoue, Saori Yamamoto, Koichiro Sugimura …

Genetics in medicine : official journal of the American College of Medical Genetics 21(8) 1900-1900 2019年8月査読有り
Correction: Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis.

Hiroki Maruyama, Kaori Miyata, Mariko Mikame, Atsumi Taguchi, Chu Guili, Masaru Shimura, Kei Murayama, Takeshi Inoue, Saori Yamamoto, Koichiro Sugimura …

Genetics in medicine : official journal of the American College of Medical Genetics 21(2) 512-515 2019年2月査読有り
Screening for Fabry Disease in Japanese Patients with Young-Onset Stroke by Measuring α-Galactosidase A and Globotriaosylsphingosine.

Kinoshita N, Hosomi N, Matsushima H, Nakamori M, Yagita Y, Yamawaki T, Torii T, Kitamura T, Sueda Y, Shimomura R …

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association 27(12) 3563-3569 2018年9月査読有り
Medullary thick ascending limb impairment in the Gla^tmTg(CAG-A4GALT) Fabry model mice.

Maruyama H, Taguchi A, Nishikawa Y, Guili C, Mikame M, Nameta M, Yamaguchi Y, Ueno M, Imai N, Ito Y …

FASEB journal : official publication of the Federation of American Societies for Experimental Biology 32(8) 4544-4559 2018年8月査読有り
Effectiveness of plasma lyso-Gb3 as a biomarker for selecting high-risk patients with Fabry disease from multispecialty clinics for genetic analysis.

Maruyama H, Miyata K, Mikame M, Taguchi A, Guili C, Shimura M, Murayama K, Inoue T, Yamamoto S, Sugimura K …

Genetics in medicine : official journal of the American College of Medical Genetics 21(1) 44-52 2018年3月査読有り
A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis

Atsumi Taguchi, Hiroki Maruyama, Masaaki Nameta, Tadashi Yamamoto, Junichiro Matsuda, Ashok B. Kulkarni, Hidekatsu Yoshioka, Satoshi Ishii

Biochemical Journal 456(3) 373-383 2013年12月15日査読有り
Screening of male dialysis patients for fabry disease by plasma globotriaosylsphingosine

Hiroki Maruyama, Takuma Takata, Yutaka Tsubata, Ryushi Tazawa, Kiyoe Goto, Jun Tohyama, Ichiei Narita, Hidekatsu Yoshioka, Satoshi Ishii

Clinical Journal of the American Society of Nephrology 8(4) 629-636 2013年4月5日査読有り
Pharmacological chaperone therapy for Fabry disease

Satoshi Ishii

PROCEEDINGS OF THE JAPAN ACADEMY SERIES B-PHYSICAL AND BIOLOGICAL SCIENCES 88(1) 18-30 2012年1月査読有り
Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha 1,4-galactosyltransferase and a mouse model for treating Fabry disease

Chikara Shiozuka, Atsumi Taguchi, Junichiro Matsuda, Yoko Noguchi, Takanori Kunieda, Kozue Uchio-Yamada, Hidekatsu Yoshioka, Ryoji Hamanaka, Shinji Yano, Shigeo Yokoyama …

JOURNAL OF BIOCHEMISTRY 149(2) 161-170 2011年2月査読有り
Preclinical Efficacy and Safety of 1-Deoxygalactonojirimycin in Mice for Fabry Disease

Satoshi Ishii, Hui-Hwa Chang, Hidekatsu Yoshioka, Tatsuo Shimada, Kazuaki Mannen, Yasunori Higuchi, Atsumi Taguchi, Jian-Qiang Fan

JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS 328(3) 723-731 2009年3月査読有り
Rescue of mutant α-galactosidase A in the endoplasmic reticulum by 1-doxygalactonojirimycin leads to trafficking to lysosomes.

R.HAMANAKA, T.SHINOHARA, S.YANO, M.NAKAMURA, A.YASUDA, S.YOKOYAMA, J.-Q.FAN, K.KAWASAKI, M.WATANABE, S.ISHII

Biochim. Biophys. Acta 1782(6) 408-413 2008年6月査読有り
Novel mutations of the GLA gene in Japanese patients with Fabry disease and their functional characterization by active site specific chaperone

Masaaki Shimotori, Hiroki Maruyama, Gen Nakamura, Takayuki Suyama, Fumiko Sakamoto, Masaaki Itoh, Shigeaki Miyabayashi, Takahiro Ohnishi, Norio Sakai, Mari Wataya-Kaneda …

HUMAN MUTATION 29(2) 331-331 2008年2月査読有り
Naked plasmid DNA-based alpha-galactosidase A gene transfer partially reduces systemic accumulation of globotriaosylceramide in Fabry mice

Gen Nakamura, Hiroki Maruyama, Satoshi Ishii, Masaaki Shimotori, Shigemi Kameda, Toru Kono, Jun-Ichi Miyazaki, Ashok B. Kulkarni, Fumitake Gejyo

MOLECULAR BIOTECHNOLOGY 38(2) 109-119 2008年2月査読有り
Active-site-specific chaperone therapy for Fabry disease: Yin and Yang of enzyme inhibitors.

J.-Q.FAN, S.ISHII

FEBS J. 274(19) 4962-4971 2007年10月査読有り
Mutant α-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin.

S.ISHII, H.-H.CHANG, K.KAWASAKI, K.YASUDA, H.-L.WU, S.C.GARMAN, J.-Q.FAN

Biochem. J. 406(2) 285-295 2007年9月査読有り
Transgenic mouse expressing human mutant α-galactosidase A in an endogenous enzyme deficient background: a biochemical animal model for a studying active-site specific chaperone therapy for Fabry disease.

S.ISHII, H.YOSHIOKA, K.MANNEN, A.B.KULKARNI, J.-Q.FAN

Biochim. Biophys. Acta 1690(3) 250-257 2004年11月査読有り
Efficient and rapid purification of recombinant human alpha-galactosidase A by affinity column chromatography

K Yasuda, HH Chang, HL Wu, S Ishii, JQ Fan

PROTEIN EXPRESSION AND PURIFICATION 37(2) 499-506 2004年10月査読有り
Cell-based screening of active-site specific chaperone for the treatment of Fabry disease

J.-Q.FAN, S.ISHII

Methods Enzymol 363 412-420 2003年査読有り
Altenative splicing in the α-galactosidase A gene: increased exon inclusion results in the Fabry cardiac phenotype

S.ISHII, S.NAKAO, R.MINAMIKAWA-TACHINO, R.J.DESNICK, J.-Q.FAN

Am. J. Hum. Genet. 70(4) 994-1002 2002年4月査読有り
Accelerated transport and maturation of lysosomal α-galactosidase A in Fabry lymphoblasts by an enzyme inhibitor

J.-Q.FAN, S.ISHII, N.ASANO, Y.SUZUKI

Nat. Med. 5(1) 112-115 1999年

MISC

In vitro inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives

N Asano, S Ishii, H Kizu, K Ikeda, K Yasuda, A Kato, OR Martin, JQ Fan

EUROPEAN JOURNAL OF BIOCHEMISTRY 267(13) 4179-4186 2000年7月
Role of Ser-65 in the activity of α-galactosidase A : characterization of a point mutation(S65T) detected in a patient with Fabry disease

Arch. Biochem. Biophys. 377(2) 228-233 2000年
α-Galactosidase transgenic mouse: heterogeneous gene expression and posttranslational glycosylation in tissues.

Glycoconj. J. 15(6) 591-594 1998年

所属学協会

共同研究・競争的資金等の研究課題

Chaperone therapy for Fabry disease

1998年 - 2008年
Gene therapy for Fabry disease

Grant-in-Aid for Scientific Research 2002年 - 2005年

石井達

基本情報

研究キーワード

研究分野

経歴

学歴

受賞

論文

MISC

所属学協会

共同研究・競争的資金等の研究課題

産業財産権

メニュー