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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...
Autores principales: | Rai, Myriam, Soragni, Elisabetta, Chou, C. James, Barnes, Glenn, Jones, Steve, Rusche, James R., Gottesfeld, Joel M., Pandolfo, Massimo |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2010
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Materias: | |
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 |
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