COA6
Description
The COA6 (cytochrome c oxidase assembly factor 6) is a protein-coding gene located on chromosome 1.
COA6 (Cytochrome c oxidase assembly factor 6) is a protein encoded by the COA6 gene in humans. It plays a critical role in the assembly of mitochondrial respiratory chain Complex IV (cytochrome c oxidase). This complex is responsible for the transfer of electrons from cytochrome c to molecular oxygen, contributing to the proton gradient that drives ATP synthesis. COA6 acts as an assembly factor, stabilizing newly formed COX2 and participating in the mitochondrial copper relay system. Mutations in the COA6 gene lead to fatal infantile cardioencephalomyopathy. The COA6 protein is located in the intermembrane space of mitochondria and interacts with proteins like SCO2 and COX2. The gene is located on chromosome 1 and encodes a 125 amino acid protein with a molecular weight of 14.1 kDa. COA6 contains a CX9CXnCX10C motif and a CHCH domain, suggesting its role as a redox protein rather than a copper metallochaperone.
COA6 plays a crucial role in the maturation of the mitochondrial respiratory chain complex IV subunit MT-CO2/COX2, potentially regulating the initial steps of complex IV assembly. Complex IV, also known as cytochrome c oxidase, is essential for the respiratory chain, catalyzing the transfer of electrons from intermembrane space cytochrome c to molecular oxygen in the mitochondrial matrix. This process contributes to the proton gradient that drives ATP synthesis. COA6 may also be necessary for the efficient formation of respiratory supercomplexes, which include complexes III and IV.
COA6 is also known as C1orf31, CEMCOX4, MC4DN13.
