Cytochrome c oxidase assembly protein COX16 homolog, mitochondrial

Definition:

Required for the assembly of the mitochondrial respiratory chain complex IV (CIV), also known as cytochrome c oxidase. Promotes the insertion of copper into the active site of cytochrome c oxidase subunit II (MT-CO2/COX2). Interacts specifically with newly synthesized MT-CO2/COX and its copper center-forming metallochaperones SCO1, SCO2 and COA6. Probably facilitates MT-CO2/COX2 association with the MITRAC assembly intermediate containing MT-CO1/COX1, thereby participating in merging the MT-CO1/COX1 and MT-CO2/COX2 assembly lines.

References:

[1]. Abhishek Aich, et al. COX16 promotes COX2 metallation and assembly during respiratory complex IV biogenesis. Elife. 2018 Jan 30;7:e32572. [Content Brief]

[2]. Cristina Cerqua, et al. COX16 is required for assembly of cytochrome c oxidase in human cells and is involved in copper delivery to COX2. Biochim Biophys Acta Bioenerg. 2018 Apr;1859(4):244-252. [Content Brief]

[3]. Liesbeth T M Wintjes, et al. A novel variant in COX16 causes cytochrome c oxidase deficiency, severe fatal neonatal lactic acidosis, encephalopathy, cardiomyopathy, and liver dysfunction. Hum Mutat. 2021 Feb;42(2):135-141. [Content Brief]

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