COX6C

Chr 8q22.2

cytochrome c oxidase subunit 6C

MANE:: ENST00000520468.7

OMIM: 124090 ↗NCBI: 1345 ↗Ensembl ↗

Annotations refreshed 9 hours ago.

Predicted protein structure

AlphaFold predicted structure

COX6C · P09669

v62025-08-01

Predicted Aligned Error matrix for COX6C — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

94.8/ 100

Very high

75 residues

Confidence breakdown

Very high(≥ 90)89%
Confident(70–90)8%
Low(50–70)3%
Very low(< 50)0%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Moderate Evidence (Amber)

Mitochondrial disorder with complex IV deficiency
Unknown
Possible mitochondrial disorder - nuclear genes
Unknown
Likely inborn error of metabolism
Unknown
Mitochondrial disorders

Disease associations (Open Targets)

neurodegenerative disease
0.55
diabetes mellitus
0.23
small cell lung carcinoma
0.21
breast carcinoma
0.20
cutaneous melanoma
0.19
esophageal squamous cell carcinoma
0.19
lung adenocarcinoma
0.19
esophageal adenocarcinoma
0.19
hepatocellular carcinoma
0.19
acute myeloid leukemia
0.19

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Cytochrome c oxidase subunit 6C

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