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SURF1 is a protein localized to the inner mitochondrial membrane and thought to be involved in the biogenesis of the cytochrome c oxidase complex. The protein is a member of the SURF1 family, which includes the related yeast protein SHY1 and rickettsial protein RP733. The gene is located in the surfeit gene cluster, a group of very tightly linked genes that do not share sequence similarity, where it shares a bidirectional promoter with SURF2 on the opposite strand. Defects in this gene are a cause of Leigh syndrome, a severe neurological disorder that is commonly associated with systemic cytochrome c oxidase deficiency.
Gene Name: | surfeit 1 |
Synonyms: | SURF1, Surfeit 1, Surfeit locus protein 1, SURF-1 |
Target Sequences: | NM_003172 NP_003163.1 Q15526 |
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