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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...
Autores principales: | Sendino Garví, Elena, Masereeuw, Rosalinde, Janssen, Manoe J. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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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 |
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