Immunogen sequence: TREEVDQVAR AYRVYYSPGP KDEDEDYIVD HTIIMYLIGP DGEFLDYFGQ NKRKGEIAAS IATHMRPYR
Highest antigen sequence identity to the following orthologs: Mouse - 86%, Rat - 86%.
Synthesis of cytochrome c oxidase 1 was initially identified in yeast as one of two cytochrome c oxidase (COX) assembly proteins that enable the assembly of cytochrome c holoenzyme, a complex that catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. Like their yeast homologs, the function of both SCO1 and SCO2 are dependent on copper ion binding. Mutations in either gene can lead to cytochrome c oxidase respiratory chain defects, with a missense mutation in human SCO1 (P174L) associated with a fatal neonatal hepatopathy when the second allele is also non-functional, suggesting the pathology is due to loss of function. It has been suggested that this mutation alters the SCO1 affinity for the copper (I) ion, thus impairing the efficiency of copper transfer to the cytochrome c oxidase.
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Protein Aliases: Protein SCO1 homolog, mitochondrial; SCO cytochrome oxidase deficient homolog 1
Gene Aliases: SCO1; SCOD1
UniProt ID: (Human) O75880
Entrez Gene ID: (Human) 6341