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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Detalles Bibliográficos
Autores principales: Rai, Myriam, Soragni, Elisabetta, Jenssen, Kai, Burnett, Ryan, Herman, David, Coppola, Giovanni, Geschwind, Daniel H., Gottesfeld, Joel M., Pandolfo, Massimo
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2008
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2373517/
https://www.ncbi.nlm.nih.gov/pubmed/18463734
http://dx.doi.org/10.1371/journal.pone.0001958

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2373517/
https://www.ncbi.nlm.nih.gov/pubmed/18463734
http://dx.doi.org/10.1371/journal.pone.0001958

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