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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Main Authors: | 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 |
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Format: | article |
Language: | EN |
Published: |
Public Library of Science (PLoS)
2013
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Subjects: | |
Online Access: | https://doaj.org/article/e476685343814a1385f90b64d0282de0 |
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