Friedreich’s ataxia variants I154F and W155R diminish frataxin-based activation of the iron-sulfur cluster assembly complex

Biochemistry, Just Accepted Manuscript, DOI: 10.1021/bi200666h
Publication Date (Web): June 14, 2011

Chi-Lin Tsai , Jennifer Bridwell-Rabb , and David P. Barondeau

Keywords: Friedreich’s ataxia (FRDA), frataxin (Fxn), GAA expansion, missense mutation, FRDA I154F, W155R variants, Nfs1, Isd11, Isu2, Fe-S cluster assembly machine, Fxn triggers sulfur transfer from Nfs1 to Isu.