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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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Detalles Bibliográficos
Autores principales: Zhang, Jinzhong, He, Jing, Johnson, Jennifer L., Rahman, Farhana, Gavathiotis, Evripidis, Cuervo, Ana Maria, Catz, Sergio D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367655/
https://www.ncbi.nlm.nih.gov/pubmed/30774622
http://dx.doi.org/10.3389/fendo.2019.00021