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Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia

Friedreich ataxia is a neurodegenerative disease caused by a GAA triplet repeat expansion in the first intron of the frataxin gene, which results in reduced expression levels of the corresponding protein. Despite numerous animal and cellular models, therapeutic options that mechanistically address i...

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Détails bibliographiques
Publié dans:Biol Open
Auteurs principaux: Poburski, Dörte, Boerner, Josefine Barbara, Koenig, Michel, Ristow, Michael, Thierbach, René
Format: Artigo
Langue:Inglês
Publié: The Company of Biologists Ltd 2016
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC4874353/
https://ncbi.nlm.nih.gov/pubmed/27106929
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/bio.017004
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