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Frataxin and mitochondrial FeS cluster biogenesis Stemmler TL; Lesuisse E; Pain D; Dancis AJ Biol Chem 2010[Aug]; 285 (35): 26737-26743Friedreich 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.|Animals[MESH]|Carbon-Sulfur Lyases/genetics/metabolism[MESH]|Frataxin[MESH]|Friedreich Ataxia/genetics/metabolism[MESH]|Humans[MESH]|Iron-Binding Proteins/genetics/*metabolism[MESH]|Iron-Sulfur Proteins/genetics/*metabolism[MESH]|Iron/*metabolism[MESH]|Mitochondria/genetics/*metabolism[MESH]|Mitochondrial Proteins/genetics/*metabolism[MESH]|Sulfides/*metabolism[MESH] |
