Ubiquinol Cytochrome C Reductase, Rieske Iron Sulfur Polypeptide 1 (UQCRFS1)

The Rieske iron-sulfur protein is a nuclear-encoded subunit of the mammalian cytochrome bc1 complex (complex III) of the mitochondrial respiratory chain. This complex, which transfers electrons from ubiquinol to cytochrome c, has 1 mitochondrial-encoded subunit (cytochrome b; MTCYB) and 9 to 10 subunits encoded by the nuclear genome. UQCRFS1 partial cDNA was derived from either chromosome 19 or chromosome 22. By screening a human chromosome 19-specific genomic cosmid library with a probe from this cDNA sequence, they identified a corresponding cosmid. Portions of this cosmid were sequenced directly. The exon, exon:intron junction, and flanking sequences verified that this cosmid, indeed, contained the genomic locus. Fluorescence in situ hybridization localized the cosmid to 19q12.