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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| Auteurs principaux: | , , , , , , , , , , , |
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| Format: | article |
| Langue: | EN |
| Publié: |
Public Library of Science (PLoS)
2013
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| Sujets: | |
| Accès en ligne: | https://doaj.org/article/e476685343814a1385f90b64d0282de0 |
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