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Bioengineered Cystinotic Kidney Tubules Recapitulate a Nephropathic Phenotype

Nephropathic cystinosis is a rare and severe disease caused by disruptions in the CTNS gene. Cystinosis is characterized by lysosomal cystine accumulation, vesicle trafficking impairment, oxidative stress, and apoptosis. Additionally, cystinotic patients exhibit weakening and leakage of the proximal...

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Detalles Bibliográficos
Autores principales: Sendino Garví, Elena, Masereeuw, Rosalinde, Janssen, Manoe J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8750898/
https://www.ncbi.nlm.nih.gov/pubmed/35011739
http://dx.doi.org/10.3390/cells11010177