cytochrome c oxidase subunit 1; cytochrome c oxidase polypeptide I (MT-CO1 COI COXI MTCO1)

Function: - subunit of cytochrome c oxidase, the component of the respiratory chain that catalyzes the reduction of oxygen to water - subunits 1-3 form the functional core of the enzyme complex. - MT-CO1 is the catalytic subunit of the enzyme - electrons originating in cytochrome c are transferred via the copper A center of subunit 2 & heme A of subunit 1 to the bimetallic center formed by heme A3 & copper B - energy metabolism; oxidative phosphorylation 4 ferrocytochrome c + O₂ + 4 H⁺ = 4 ferricytochrome c + 2 H₂O Structure: - belongs to the heme-copper respiratory oxidase family Compartment: - mitochondrial inner membrane; multi-pass membrane protein Pathology: - defects in MT-CO1 are a cause of a) Leber hereditary optic neuropathy (LHON) b) acquired idiopathic sideroblastic anemia c) cytochrome c oxidase deficiency d) recurrent myoglobinuria mitochondrial e) mitochondrial sensorineural deafness f) colorectal cancer (CRC) Laboratory: - MT-CO1 gene mutation

General

cytochrome c oxidase subunit enzyme catalytic subunit mitochondrial membrane protein transmembrane 12 protein

Properties

SIZE: entity length = 513 aa MW = 57 kD COMPARTMENT: mitochondria MOTIF: Mitochondrial matrix {1-19} transmembrane domain {20-42} Mitochondrial intermembrane {43-56} transmembrane domain {57-79} MOTIF: Iron [Fe]-binding site SITE: 61-61 Mitochondrial matrix {80-99} transmembrane domain {100-122} Mitochondrial intermembrane {123-146} transmembrane domain {147-169} Mitochondrial matrix {170-181} transmembrane domain {182-204} Mitochondrial intermembrane {205-235} transmembrane domain {236-258} MOTIF: copper [Cu]-binding site SITE: 240-240 MOTIF: histidine residue (3) copper [Cu]-binding site SITE: 244-244 MOTIF: histidine residue (3) Mitochondrial matrix {259-270} transmembrane domain {271-293} MOTIF: copper [Cu]-binding site SITE: 290-290 MOTIF: histidine residue (3) copper [Cu]-binding site SITE: 291-291 MOTIF: histidine residue (3) Mitochondrial intermembrane {294-302} transmembrane domain {303-325} Mitochondrial matrix {326-336} transmembrane domain {337-359} Mitochondrial intermembrane {360-373} transmembrane domain {374-396} MOTIF: Iron [Fe]-binding site SITE: 376-376 Iron [Fe]-binding site SITE: 378-378 Mitochondrial matrix {397-408} transmembrane domain {409-431} Mitochondrial intermembrane {432-450} transmembrane domain {451-473} Mitochondrial matrix {474-513}

Database Correlations

OMIM correlations UniProt P00395 Pfam PF00115 Entrez Gene 4512 Kegg hsa:4512 ENZYME 1.9.3.1