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Generation and Characterisation of Friedreich Ataxia YG8R Mouse Fibroblast and Neural Stem Cell Models

BACKGROUND: Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disease caused by GAA repeat expansion in the first intron of the FXN gene, which encodes frataxin, an essential mitochondrial protein. To further characterise the molecular abnormalities associated with FRDA pathogenes...

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Detalles Bibliográficos
Autores principales: Sandi, Chiranjeevi, Sandi, Madhavi, Jassal, Harvinder, Ezzatizadeh, Vahid, Anjomani-Virmouni, Sara, Al-Mahdawi, Sahar, Pook, Mark A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3931792/
https://www.ncbi.nlm.nih.gov/pubmed/24586819
http://dx.doi.org/10.1371/journal.pone.0089488