Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly.

J Biol Chem. 2010 Oct 2. [Epub ahead of print]

Gakh O, Bedekovics T, Duncan SF, Smith DY 4th, Berkholz DS, Isaya G.
Mayo Clinic, United States.

Keywords: frataxin (FXN), Fe-S cluster, FXN mRNA, FXN42-210, FXN81-210, levels of both isoforms are relevant, FXN81-210/FXN42-210 molar ratio, replacement therapies.