Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice

Fabry disease is an X-linked metabolic disorder caused by a deficiency of α-galactosidase A (α-Gal A). The enzyme defect leads to the systemic accumulation of glycosphingolipids with α-galactosyl moieties consisting predominantly of globotriaosylceramide (Gb3). In patients with this disorder, glycol...

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Main Authors: Ohshima, Toshio, Schiffmann, Raphael, Murray, Gary J., Kopp, Jeffrey, Quirk, Jane M., Stahl, Stefanie, Chan, Chi-Chao, Zerfas, Patricia, Tao-Cheng, Jung-Hwa, Ward, J. M., Brady, Roscoe O., Kulkarni, Ashok B.
格式: Artigo
語言:Inglês
出版: The National Academy of Sciences 1999
主題:
Biological Sciences
在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC26897/
https://ncbi.nlm.nih.gov/pubmed/10339603
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