Article
Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues.
Human molecular genetics - 15 Nov 2007
Martelli Alain, Wattenhofer-Donzé Marie, Schmucker Stéphane, Bouvet Samuel, Reutenauer Laurence, Puccio Hélène
Abstract excerpt
Friedreich ataxia, the most common recessive ataxia, is caused by the deficiency of the mitochondrial protein frataxin (Fxn), an iron chaperone involved in the assembly of Fe-S clusters (ISC). In yeast, mitochondria play a central role for all Fe-S proteins, independently of their subcellular localization. In mammalian cells, this central role of mitochondria remains controversial as an independent cytosolic ISC...
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