Protein: Cytochrome c oxidase subunit 4 isoform 1, mitochondrial (Cytochrome c oxidase polypeptide IV) (Cytochrome c oxidase subunit IV isoform 1) (COX IV-1) (P19783)
Organism: Mus musculus (Mouse)
Function: Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.
Subcellular location: Mitochondrion inner membrane {ECO:0000269|PubMed:34616041, ECO:0000269|PubMed:37195149, ECO:0000269|PubMed:38575788}; Single-pass membrane protein {ECO:0000269|PubMed:34616041, ECO:0000269|PubMed:38575788}.
Sequence: MLASRALSLIGKRAISTSVCLRAHGSVVKSEDYAFPTYADRRDYPLPDVAHVTMLSASQKALKEKEKADWSSLSRDEKVQLYRIQFNESFAEMNRGTNEWKTVVGMAMFFIGFTALVLIWEKSYVYGPIPHTFDRDWVAMQTKRMLDMKANPIQGFSAKWDYDKNEWKK
InterPro domains: - IPR004203: Cytochrome c oxidase subunit IV family (family) [1-169]
- IPR013288: Cytochrome c oxidase subunit IV (family) [39-168]
- IPR036639: Cytochrome c oxidase subunit IV superfamily (homologous_superfamily) [23-169]
Interaction partners: - Cytochrome b-c1 complex subunit 1, mitochondrial
- Cytochrome c1, heme protein, mitochondrial
- Cytochrome c oxidase subunit 6B1
- Cytochrome c oxidase subunit 6C
- Cytochrome c oxidase subunit 1
- Cytochrome c oxidase subunit 7C, mitochondrial
- Cytochrome b-c1 complex subunit 2, mitochondrial
- Cytochrome c oxidase subunit 5A, mitochondrial
- Cytochrome c oxidase subunit 2
- Cytochrome c oxidase subunit 5B, mitochondrial
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0003824 (catalytic activity), GO:0005215 (transporter activity), GO:0022857 (transmembrane transporter activity), GO:0016491 (oxidoreductase activity), GO:0009055 (electron transfer activity), GO:0015075 (monoatomic ion transmembrane transporter activity), GO:0015318 (inorganic molecular entity transmembrane transporter activity), GO:0016675 (oxidoreductase activity, acting on a heme group of donors), GO:0022804 (active transmembrane transporter activity), GO:0015399 (primary active transmembrane transporter activity), GO:0022890 (inorganic cation transmembrane transporter activity), GO:0022853 (active monoatomic ion transmembrane transporter activity), GO:0004129 (cytochrome-c oxidase activity), GO:0008324 (monoatomic cation transmembrane transporter activity), GO:0015453 (oxidoreduction-driven active transmembrane transporter activity), GO:0015078 (proton transmembrane transporter activity)
Biological Process (BP): GO:0008150 (biological process), GO:0050896 (response to stimulus), GO:0006950 (response to stress), GO:0009628 (response to abiotic stimulus), GO:0070482 (response to oxygen levels), GO:0001666 (response to hypoxia), GO:0036293 (response to decreased oxygen levels)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0031975 (envelope), GO:0016020 (membrane), GO:0005737 (cytoplasm), GO:0031090 (organelle membrane), GO:0031967 (organelle envelope), GO:0005739 (mitochondrion), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0005740 (mitochondrial envelope), GO:0019866 (organelle inner membrane), GO:0043231 (intracellular membrane-bounded organelle), GO:0031966 (mitochondrial membrane), GO:0005743 (mitochondrial inner membrane)