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Frataxin and mitochondrial FeS cluster biogenesis
Timothy L Stemmler1, Emmanuel Lesuisse2, Debkumar Pain3
1Department of Biochemistry and Molecular Biology, Wayne State University School of Medicine, Detroit, Michigan 48201, France.
Abstract:
Friedreich ataxia is an inherited neurodegenerative disease caused by frataxin deficiency. Frataxin is a conserved mitochondrial protein that plays a role in FeS cluster assembly in mitochondria. FeS clusters are modular cofactors that perform essential functions throughout the cell. They are synthesized by a multistep and multisubunit mitochondrial machinery that includes the scaffold protein Isu for assembling a protein-bound FeS cluster intermediate. Frataxin interacts with Isu, iron, and the cysteine desulfurase Nfs1, which supplies sulfide, thus placing it at the center of mitochondrial FeS cluster biosynthesis.
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