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lüll Iron-sulfur cluster biogenesis and human disease Rouault TA; Tong WHTrends Genet 2008[Aug]; 24 (8): 398-407Iron-sulfur (Fe-S) clusters are essential for numerous biological processes, including mitochondrial respiratory chain activity and various other enzymatic and regulatory functions. Human Fe-S cluster assembly proteins are frequently encoded by single genes, and inherited defects in some of these genes cause disease. Recently, the spectrum of diseases attributable to abnormal Fe-S cluster biogenesis has extended beyond Friedreich ataxia to include a sideroblastic anemia with deficiency of glutaredoxin 5 and a myopathy associated with a deficiency of a Fe-S cluster assembly scaffold protein, ISCU. Mutations within other mammalian Fe-S cluster assembly genes could be causative for human diseases that manifest distinctive combinations of tissue-specific impairments. Thus, defects in the iron-sulfur cluster biogenesis pathway could underlie many human diseases.|ATP-Binding Cassette Transporters/genetics[MESH]|Anemia, Sideroblastic/genetics[MESH]|Frataxin[MESH]|Friedreich Ataxia/genetics[MESH]|Glutaredoxins/genetics[MESH]|Humans[MESH]|Iron-Binding Proteins/genetics[MESH]|Iron-Sulfur Proteins/*biosynthesis/*genetics[MESH]|Models, Biological[MESH]|Muscular Diseases/genetics[MESH]|Mutation[MESH] |