Proteins/UNIPROT:Q5RI15

COX20

ProteinUNIPROT:Q5RI15

Essential for the assembly of the mitochondrial respiratory chain complex IV (CIV), also known as cytochrome c oxidase. Acts as a chaperone in the early steps of cytochrome c oxidase subunit II (MT-CO2/COX2) maturation, stabilizing the newly synthesized protein and presenting it to metallochaperones SCO1/2 which in turn facilitates the incorporation of the mature MT-CO2/COX2 into the assembling CIV holoenzyme..

Function from UniProtKB (Q5RI15), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

3 rows

mitochondrial complex IV deficiency, nuclear type 11 has a germline basis in COX20DiseaseGenCC HGNC:26970|MONDO:0033645Inferred only
mitochondrial complex IV deficiency, nuclear-type has a germline basis in COX20DiseaseGenCC HGNC:26970|MONDO:0033885Inferred only
mitochondrial disease has a germline basis in COX20DiseaseGenCC HGNC:26970|MONDO:0044970Inferred only

Statements

6 statements

6 statements · 2 reference databases · Subject: COX20 · Sources: Hetionet, Reactome

Order

EGR1 interacts with COX20ProteinHetionet hetionet:interacts:116228-1958Inferred only
UBC interacts with COX20ProteinHetionet hetionet:interacts:116228-7316Inferred only
COX20 participates in Aerobic respiration and respiratory electron transportPathwayReactome R-HSA-1428517Inferred only
COX20 participates in Complex IV assemblyPathwayReactome R-HSA-9864848Inferred only
COX20 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
COX20 participates in Respiratory electron transportPathwayReactome R-HSA-611105Inferred only

Other names

2 names

Resolves to: COX20

Also called: Cytochrome c oxidase assembly protein COX20, mitochondrialFAM36A

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