Possible role of transforming growth factor-β1 and vascular endothelial growth factor in Fabry disease nephropathy.

ABSTRACT: Fabry disease is a lysosomal storage disorder (LSD) caused by deficiency of α-galactosidase A (α-gal A), resulting in deposition of globotriaosylceramide (Gb3; also known as ceramide trihexoside) in the vascular endothelium of many organs. A gradual accumulation of Gb3 leads to cardiovascular, cerebrovascular and renal dysfunction. Endothelial cell dysfunction leads to renal complications, one of the main symptoms of Fabry disease. However, the pathological mechanisms by which endothelial dysfunction occurs in Fabry disease are poorly characterized. The purpose of this study was to investigate whether the expression of transforming growth factor-β1 (TGF-β1) and vascular endothelial growth factor

SUBMITTER: Lee MH 

PROVIDER: S-EPMC4042857 | biostudies-literature | 2012 Dec

REPOSITORIES: biostudies-literature