Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model

Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model

Friedreich ataxia (FRDA) is an inherited neurodegenerative disorder caused by GAA repeat expansion within the FXN gene, leading to epigenetic changes and heterochromatin-mediated gene silencing that result in a frataxin protein deficit. Histone deacetylase (HDAC) inhibitors, including pimelic o-amin...

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Bibliographic Details
Main Authors: Chiranjeevi Sandi, Ricardo Mouro Pinto, Sahar Al-Mahdawi, Vahid Ezzatizadeh, Glenn Barnes, Steve Jones, James R. Rusche, Joel M. Gottesfeld, Mark A. Pook
Format: Article
Language:English
Published: Elsevier 2011-06-01
Series:Neurobiology of Disease
Subjects:
Friedreich ataxia
FRDA, frataxin
Trinucleotide repeat
Transgenic mouse model
Histone deacetylase inhibitor
HDAC inhibitor
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996111000702

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