Bioengineered Cystinotic Kidney Tubules Recapitulate a Nephropathic Phenotype

Bioengineered Cystinotic Kidney Tubules Recapitulate a Nephropathic Phenotype

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

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Bibliographic Details
Main Authors: Elena Sendino Garví, Rosalinde Masereeuw, Manoe J. Janssen
Format: Article
Language:English
Published: MDPI AG 2022-01-01
Series:Cells
Subjects:
nephropathic cystinosis
lysosomal storage disease
hollow fiber membrane
3-dimensional models
autophagy
Online Access:https://www.mdpi.com/2073-4409/11/1/177

Internet

https://www.mdpi.com/2073-4409/11/1/177

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