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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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Hlavní autoři: Shen, Jin-Song, Meng, Xing-Li, Moore, David F., Quirk, Jane M., Shayman, James A., Schiffmann, Raphael, Kaneski, Christine R.
Médium: Artigo
Jazyk:Inglês
Vydáno: 2008
Témata:
On-line přístup: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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