Mannose 6-phosphate receptor and sortilin mediated endocytosis of α-galactosidase A in kidney endothelial cells.

Prominent vasculopathy in Fabry disease patients is caused by excessive intracellular accumulation of globotriaosylceramide (GL-3) throughout the vascular endothelial cells causing progressive cerebrovascular, cardiac and renal impairments. The vascular lesions lead to myocardial ischemia, atherogen...

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Bibliographic Details
Main Authors: Thaneas Prabakaran, Rikke Nielsen, Simon C Satchell, Peter W Mathieson, Ulla Feldt-Rasmussen, Søren S Sørensen, Erik I Christensen
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2012-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3386966?pdf=render