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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2013
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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 |
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