Mechanism of action of 2-aminobenzamide HDAC inhibitors in reversing gene silencing in Friedreich’s ataxia

The genetic defect in Friedreich’s ataxia (FRDA) is the hyperexpansion of a GAA•TTC triplet in the first intron of the FXN gene, encoding the essential mitochondrial protein frataxin. Histone posttranslational modifications near the expanded repeats are consistent with heterochromatin formation and...

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Bibliographic Details
Main Authors: Elisabetta eSoragni, C. James Chou, James R. Rusche, Joel M. Gottesfeld
Format: Article
Language:English
Published: Frontiers Media S.A. 2015-03-01
Series:Frontiers in Neurology
Subjects:
Online Access:http://journal.frontiersin.org/Journal/10.3389/fneur.2015.00044/full