Friedreich’s Ataxia reveals a mechanism for coordinate regulation of oxidative metabolism via feedback inhibition of the SIRT3 deacetylase

Hum. Mol. Genet. (2012) doi: 10.1093/hmg/dds095 First published online: March 6, 2012

Gregory R. Wagner 1, P. Melanie Pride 2, Clifford M. Babbey 2 and R. Mark Payne1,2

1 Dept. of Medical & Molecular Genetics, 2D ept. of Pediatrics, Riley Heart Research Center, Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN 46202

Keywords: Friedreich’s Ataxia (FRDA), frataxin, lysine acetylation, mitochondrial function, SIRT3 deacetylase, protein hyperacetylation.