A TAT-Frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich’s Ataxia mouse model

Hum. Mol. Genet. (2011) doi: 10.1093/hmg/ddr554

Piyush M. Vyas, Wendy J. Tomamichel, P. Melanie Pride, Clifford M. Babbey, Qiujuan Wang, Jennifer Mercier, Elizabeth M. Martin, and R. Mark Payne

Keywords:Friedreich’s Ataxia (FRDA), frataxin, iron-sulfur (Fe-S) cluster, progressive ataxia, fatal cardiomyopathy, TAT-Frataxin (TAT-FXN) fusion protein, reduced caspase 3 activation, iron oxidant stress, aconitase, heart, protein replacement therapy.

Cardiomyopathy in Friedreich’s Ataxia

Acta Neurologica Belgica, N° 3 (Vol. 111/3) p.183-187, 2011.

Faisal Rahman and Massimo Pandolfo
The John Radcliffe Hospital, Oxford, UK; Service de Neurologie, Hôpital Erasme - Université Libre de Bruxelles, Brussels, Belgium

Keywords: Friedreich’s ataxia (FRDA), spinocerebellar degeneration, cardiomyopathy, frataxin, iron, iron-sulphur clusters, oxidative stress.