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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Main Authors: Daniel Fil (Author), Balu K. Chacko (Author), Robbie Conley (Author), Xiaosen Ouyang (Author), Jianhua Zhang (Author), Victor M. Darley-Usmar (Author), Aamir R. Zuberi (Author), Cathleen M. Lutz (Author), Marek Napierala (Author), Jill S. Napierala (Author)
Format: Book
Published: The Company of Biologists, 2020-07-01T00:00:00Z.
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