AlphaFold predicted structure
MT-CO3 · P00414
Mean pLDDT
97.9/ 100
Very high
261 residues
Confidence breakdown
- Very high(≥ 90)99%
- Confident(70–90)1%
- Low(50–70)0%
- Very low(< 50)0%
Open interactive 3D viewer
AlphaFold (Jumper et al., 2021) · CC BY 4.0
MT-CO3
Chr mitochondriamitochondrially encoded cytochrome c oxidase III
- Aliases:
- COX3, COIII, CO3
Annotations refreshed 10 hours ago.
Predicted protein structure
Clinical relevance (Genomics England PanelApp)
Diagnostic Grade (Green)
Likely inborn error of metabolism
MITOCHONDRIALMitochondrial disorders
MITOCHONDRIALUndiagnosed metabolic disorders
MITOCHONDRIALAlbinism or congenital nystagmus
MITOCHONDRIALEarly onset or syndromic epilepsy
MITOCHONDRIALInfantile nystagmus
MITOCHONDRIALChildhood onset dystonia, chorea or related movement disorder
MITOCHONDRIAL
Disease associations (Open Targets)
mitochondrial disease
0.67leigh syndrome due to mitochondrial complex iv deficiency
0.63Isolated cytochrome C oxidase deficiency
0.63Leber hereditary optic neuropathy
0.62MELAS syndrome
0.57MERRF
0.52MERRF syndrome
0.52Mitochondrial myopathy
0.49mitochondrial non-syndromic sensorineural hearing loss
0.49inborn mitochondrial myopathy
0.49
Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.
Protein function (UniProt)
Cytochrome c oxidase subunit 3
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
Diseases associated with MT-CO3
Curated MONDO disease pages that list MT-CO3 among their top associated genes.