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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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Detalles Bibliográficos
Autores principales:	Fil, Daniel, Chacko, Balu K., Conley, Robbie, Ouyang, Xiaosen, Zhang, Jianhua, Darley-Usmar, Victor M., Zuberi, Aamir R., Lutz, Cathleen M., Napierala, Marek, Napierala, Jill S.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	The Company of Biologists Ltd 2020
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7406325/ https://www.ncbi.nlm.nih.gov/pubmed/32586831 http://dx.doi.org/10.1242/dmm.045229

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