Target intelligence / Profile preview

Cytochrome c oxidase assembly factor 4 homolog (COA4)

Target: COA4
Molecular classification: Mitochondrial assembly factor, Accessory protein of electron transport chain Complex IV, Sometimes described as a "twin CX9C protein"

Overview

Cytochrome c oxidase assembly factor 4 homolog (COA4) is an accessory mitochondrial protein critical for the assembly of cytochrome c oxidase (Complex IV), the terminal enzyme in the electron transport chain. COA4 is required for correct biogenesis of Complex IV and thereby for efficient mitochondrial respiration and ATP production. Mutations or deficiencies in COA4 disrupt oxidative phosphorylation, leading to mitochondrial disorders including cardio- and encephalomyopathies. COA4 functions as part of several macromolecular assemblies with other metallochaperones and twin CX9C proteins to coordinate metal center incorporation during cytochrome c oxidase biogenesis.

Other names: COA4CHCHD8CMC3E2IG2

Mechanism of action

Not applicable. COA4 is not a therapeutic target for currently available drugs.

Biological functions

Promotes assembly and stability of cytochrome c oxidase (Complex IV) in the mitochondrial inner membraneNecessary for mitochondrial oxidative phosphorylationSupports electron transport and cellular respiration

Disease associations

Mitochondrial disordersCardiomyopathy (due to cytochrome c oxidase deficiency)Encephalomyopathy

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