HDAC Inhibitors Correct Frataxin Deficiency in a Friedreich Ataxia Mouse Model

BACKGROUND: Friedreich ataxia, an autosomal recessive neurodegenerative and cardiac disease, is caused by abnormally low levels of frataxin, an essential mitochondrial protein. All Friedreich ataxia patients carry a GAA⋅TTC repeat expansion in the first intron of the frataxin gene, either in the hom...

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Détails bibliographiques
Auteurs principaux: Rai, Myriam, Soragni, Elisabetta, Jenssen, Kai, Burnett, Ryan, Herman, David, Coppola, Giovanni, Geschwind, Daniel H., Gottesfeld, Joel M., Pandolfo, Massimo
Format: Artigo
Langue:Inglês
Publié: Public Library of Science 2008
Sujets:
Research Article
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC2373517/
https://ncbi.nlm.nih.gov/pubmed/18463734
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0001958
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