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Globotriaosylceramide induces oxidative stress and up-regulates cell adhesion molecule expression in Fabry disease endothelial cells

Fabry disease, an X-linked systemic vasculopathy, is caused by a deficiency of α-galactosidase A resulting in globotriaosylceramide (Gb(3)) storage in cells. The pathogenic role of Gb(3) in the disease is not known. Based on previous work, we tested the hypothesis that accumulation of Gb(3) in the v...

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Detalhes bibliográficos
Main Authors: Shen, Jin-Song, Meng, Xing-Li, Moore, David F., Quirk, Jane M., Shayman, James A., Schiffmann, Raphael, Kaneski, Christine R.
Formato: Artigo
Idioma:Inglês
Publicado em: 2008
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2593623/
https://ncbi.nlm.nih.gov/pubmed/18707907
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2008.06.016
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