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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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Detalhes bibliográficos
Main Authors: Sandi, Chiranjeevi, Sandi, Madhavi, Jassal, Harvinder, Ezzatizadeh, Vahid, Anjomani-Virmouni, Sara, Al-Mahdawi, Sahar, Pook, Mark A.
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2014
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3931792/
https://ncbi.nlm.nih.gov/pubmed/24586819
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0089488
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