Mitochondrial Dynamics of Proximal Tubular Epithelial Cells in Nephropathic Cystinosis

Mitochondrial Dynamics of Proximal Tubular Epithelial Cells in Nephropathic Cystinosis

Nephropathic cystinosis is a rare lysosomal storage disorder caused by mutations in <i>CTNS</i> gene leading to Fanconi syndrome. Independent studies reported defective clearance of damaged mitochondria and mitochondrial fragmentation in cystinosis. Proteins involved in the mitochondrial...

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Bibliographic Details
Main Authors: Domenico De Rasmo, Anna Signorile, Ester De Leo, Elena V. Polishchuk, Anna Ferretta, Roberto Raso, Silvia Russo, Roman Polishchuk, Francesco Emma, Francesco Bellomo
Format: Article
Language:English
Published: MDPI AG 2019-12-01
Series:International Journal of Molecular Sciences
Subjects:
fanconi syndrome
nephropathic cystinosis
mitochondrial dynamics
cysteamine
mitochondrial fusion
mitochondrial fission
mitochondrial cristae
Online Access:https://www.mdpi.com/1422-0067/21/1/192

Internet

https://www.mdpi.com/1422-0067/21/1/192

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