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Article Dans Une Revue Journal of Biological Chemistry Année : 2010

Frataxin and mitochondrial FeS cluster biogenesis.

Résumé

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.

Dates et versions

hal-00518153 , version 1 (16-09-2010)

Identifiants

Citer

Timothy L Stemmler, Emmanuel Lesuisse, Debkumar Pain, Andrew Dancis. Frataxin and mitochondrial FeS cluster biogenesis.. Journal of Biological Chemistry, 2010, 285 (35), pp.26737-43. ⟨10.1074/jbc.R110.118679⟩. ⟨hal-00518153⟩
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