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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 glycosphingo...

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Detalles Bibliográficos
Autores principales: Liebau, Max C., Braun, Fabian, Höpker, Katja, Weitbrecht, Claudia, Bartels, Valerie, Müller, Roman-Ulrich, Brodesser, Susanne, Saleem, Moin A., Benzing, Thomas, Schermer, Bernhard, Cybulla, Markus, Kurschat, Christine E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3656911/
https://www.ncbi.nlm.nih.gov/pubmed/23691056
http://dx.doi.org/10.1371/journal.pone.0063506