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論文
- タイトル
- タイトル（英）
- Immunohistochemical characterization of transgenic mice highly expressing human lysosomal α-galactosidase
- 参照URL
- https://researchmap.jp/Yonejii20030920/published_papers/38295538
- 著者
- 著者（英）
- Ryoichi Kase,Michie Shimmoto,Kohji Itoh,Kouichi Utsumi,Masaharu Kotani,Choji Taya,Hiromichi Yonekawa,Hitoshi Sakuraba
- 担当区分
- 概要
- 概要（英）
- Human lysosomal α-galactosidase predominantly hydrolyzes ceramide trihexoside. A transgenic mouse line, C57BL/6CrSIc-TgN(GLA) 1951 Rin, highly expressing human α-galactosidase, has been established and investigated biochemically and immunohistochemically in order to clarify the distribution of the expressed enzyme proteins and to evaluate it as a donor model of organ transplantation therapy for Fabry disease caused by a genetic defect of α-galactosidase. In these transgenic mice, about five copies of the transgene were integrated, and α-galactosidase activity was expressed in liver, kidney, heart, spleen, small intestine, submaxillary gland, skeletal muscle, cerebrum, cerebellum, bone marrow cells and serum. The enzyme activity was about 22 to 11,080-fold higher than that in non-transgenic mice. In liver, heart and kidney tissues, which are important organs for transplantation studies, sufficient amounts of α-galactosidase mRNAs were transcribed, and the expressed enzymes, with molecular weights of 54-60 kDa, are abundant in the liver (enzyme activity: 53,965 nmol h-1 mg-1 protein) and heart (39,906 nmol h-1 mg-1 protein), followed by in the kidney tissue (9177 nmol h-1 mg-1 protein), respectively. An immunohistochemical microscopic study clearly demonstrated the distribution of the expressed enzyme proteins in kidney and liver tissues. Highly expressed α-galactosidase was detected in glomerular cells, tubular cells and hepatocytes. These transgenic mice will be useful as a donor model for experimental organ transplantation, and also it will enable recurrent biopsies and long-term observation. The organ transplantation data on mice will provide us with important information. Copyright (C) 1998 Elsevier Science B.V.
- 出版者・発行元
- 出版者・発行元（英）
- 誌名
- 誌名（英）
- Biochimica et Biophysica Acta - Molecular Basis of Disease
- 巻
- 1406
- 号
- 3
- 開始ページ
- 260
- 終了ページ
- 266
- 出版年月
- 1998年4月28日
- 査読の有無
- 査読有り
- 招待の有無
- 掲載種別
- 研究論文（学術雑誌）
- ISSN
- 0925-4439
- DOI URL
- https://doi.org/10.1016/S0925-4439(98)00012-X
- 共同研究・競争的資金等の研究課題
研究者
米川 博通
( )