Wednesday, February 10, 2016

Human Frataxin Folds Via an Intermediate State. Role of the C-Terminal Region

Santiago E. Faraj, Rodolfo M. González-Lebrero, Ernesto A. Roman & Javier Santos; (Nature) Scientific Reports 6, Article number: 20782 (2016) doi:10.1038/srep20782

OPEN

The aim of this study is to investigate the folding reaction of human frataxin, whose deficiency causes the neurodegenerative disease Friedreich’s Ataxia (FRDA). The characterization of different conformational states would provide knowledge about how frataxin can be stabilized without altering its functionality.