Molecular control of the cytosolic aconitase/IRP1 switch by extramitochondrial frataxin.

Hum Mol Genet. 2010 Jan 6.

Condò I, Malisan F, Guccini I, Serio D, Rufini A, Testi R.

Laboratory of Signal Transduction, Department of Experimental Medicine and Biochemical Sciences, University of Rome "Tor Vergata", Rome.

Keywords: , frataxin,  Friedreich's Ataxia (FRDA),  progressive gait instability, cardiomyopathy, high incidence of diabetes, iron-binding protein,  iron-sulfur clusters (ISC),  iron-chaperone, extramitochondrial pool of mature frataxin, cytosolic aconitase/IRP1, enzymatic and a RNA-binding function,  IRP1 activation.