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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...

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Bibliografische gegevens
Gepubliceerd in:PLoS One
Hoofdauteurs: Ivanova, Margarita M., Changsila, Erk, Iaonou, Chidima, Goker-Alpan, Ozlem
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: Public Library of Science 2019
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Online toegang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6329517/
https://ncbi.nlm.nih.gov/pubmed/30633777
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0210617
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