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

Neurobiology of Disease, doi:10.1016/j.nbd.2011.02.016

Chiranjeevi Sandi a, Ricardo Mouro Pinto a, 1, Sahar Al-Mahdawi a, Vahid Ezzatizadeh a, Glenn Barnes b, Steve Jones b, James R. Rusche b, Joel M. Gottesfeld c and Mark A. Pook a

a Division of Biosciences, School of Health Sciences and Social Care, Brunel University, Uxbridge, UB8 3PH, UK
b Repligen Corporation, Waltham, MA, USA
c Department of Molecular Biology, The Scripps Research Institute, La Jolla, CA, USA
Available online 10 March 2011.

keywords: Friedreich ataxia (FRDA), Histone deacetylase (HDAC) inhibitors, pimelic o-aminobenzamide compounds 106, 109 and 136, FXN gene silencing, short-term studies, long-term therapeutic effects, FRDA mouse model.