Two New Pimelic Diphenylamide HDAC Inhibitors Induce Sustained Frataxin Upregulation in Cells from Friedreich's Ataxia Patients and in a Mouse Model

BACKGROUND: Friedreich's ataxia (FRDA), the most common recessive ataxia in Caucasians, is due to severely reduced levels of frataxin, a highly conserved protein, that result from a large GAA triplet repeat expansion within the first intron of the frataxin gene (FXN). Typical marks of heterochr...

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Detalles Bibliográficos
Autores principales: Rai, Myriam, Soragni, Elisabetta, Chou, C. James, Barnes, Glenn, Jones, Steve, Rusche, James R., Gottesfeld, Joel M., Pandolfo, Massimo
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2010
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2809102/
https://www.ncbi.nlm.nih.gov/pubmed/20098685
http://dx.doi.org/10.1371/journal.pone.0008825

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2809102/
https://www.ncbi.nlm.nih.gov/pubmed/20098685
http://dx.doi.org/10.1371/journal.pone.0008825

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