Dysregulated autophagy contributes to podocyte damage in Fabry's disease.

Fabry's disease results from an inborn error of glycosphingolipid metabolism that is due to deficiency of the lysosomal hydrolase α-galactosidase A. This X-linked defect results in the accumulation of enzyme substrates with terminally α-glycosidically bound galactose, mainly the neutral glycosp...

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Autores principales: Max C Liebau, Fabian Braun, Katja Höpker, Claudia Weitbrecht, Valerie Bartels, Roman-Ulrich Müller, Susanne Brodesser, Moin A Saleem, Thomas Benzing, Bernhard Schermer, Markus Cybulla, Christine E Kurschat
Formato: article
Lenguaje:EN
Publicado: Public Library of Science (PLoS) 2013
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Acceso en línea:https://doaj.org/article/e476685343814a1385f90b64d0282de0
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