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Impaired autophagic and mitochondrial functions are partially restored by ERT in Gaucher and Fabry diseases
The major cellular clearance pathway for organelle and unwanted proteins is the autophagy-lysosome pathway (ALP). Lysosomes not only house proteolytic enzymes, but also traffic organelles, sense nutrients, and repair mitochondria. Mitophagy is initiated by damaged mitochondria, which is ultimately d...
Autores principales: | Ivanova, Margarita M., Changsila, Erk, Iaonou, Chidima, Goker-Alpan, Ozlem |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329517/ https://www.ncbi.nlm.nih.gov/pubmed/30633777 http://dx.doi.org/10.1371/journal.pone.0210617 |
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