A Short Synthetic Peptide Mimetic of Apolipoprotein A1 Mediates Cholesterol and Globotriaosylceramide Efflux from Fabry Fibroblasts

Fabry disease is an X-linked sphingolipid storage disorder caused by a deficiency of the lysosomal enzyme α-galactosidase A (AGA, EC 3.2.1.22) resulting in the intracellular accumulation of globotriaosylceramide (Gb3). We found that Gb3 storage also correlates with accumulation of endosomal–lysosoma...

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Udgivet i:JIMD Rep
Main Authors: Schueler, Ulrike, Kaneski, Christine, Remaley, Alan, Demosky, Stephen, Dwyer, Nancy, Blanchette-Mackie, Joan, Hanover, John, Brady, Roscoe
Format: Artigo
Sprog:Inglês
Udgivet: Springer Berlin Heidelberg 2015
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5059196/
https://ncbi.nlm.nih.gov/pubmed/26683465
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/8904_2015_507
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