Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia

Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia

Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disease caused by reduced expression of the mitochondrial protein frataxin (FXN). Most FRDA patients are homozygous for large expansions of GAA repeat sequences in intron 1 of FXN, whereas a fraction of patients are compound...

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Bibliographic Details
Main Authors: Daniel Fil, Balu K. Chacko, Robbie Conley, Xiaosen Ouyang, Jianhua Zhang, Victor M. Darley-Usmar, Aamir R. Zuberi, Cathleen M. Lutz, Marek Napierala, Jill S. Napierala
Format: Article
Language:English
Published: The Company of Biologists 2020-07-01
Series:Disease Models & Mechanisms
Subjects:
friedreich's ataxia
senescence
mitochondria
frataxin
point mutation
oxidative stress
Online Access:http://dmm.biologists.org/content/13/7/dmm045229

Internet

http://dmm.biologists.org/content/13/7/dmm045229

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