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Chaperone-Mediated Autophagy Upregulation Rescues Megalin Expression and Localization in Cystinotic Proximal Tubule Cells

Cystinosis is a lysosomal storage disorder caused by defects in CTNS, the gene that encodes the lysosomal cystine transporter cystinosin. Patients with nephropathic cystinosis are characterized by endocrine defects, defective proximal tubule cell (PTC) function, the development of Fanconi syndrome a...

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Publicat a:Front Endocrinol (Lausanne)
Autors principals: Zhang, Jinzhong, He, Jing, Johnson, Jennifer L., Rahman, Farhana, Gavathiotis, Evripidis, Cuervo, Ana Maria, Catz, Sergio D.
Format: Artigo
Idioma:Inglês
Publicat: Frontiers Media S.A. 2019
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6367655/
https://ncbi.nlm.nih.gov/pubmed/30774622
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fendo.2019.00021
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