Involved in several processes, including mitochondrial cytochrome c oxidase assembly; positive regulation of cytochrome-c oxidase activity; and protein stabilization. Acts upstream of or within intrinsic apoptotic signaling pathway. Located in mitochondrion. Human ortholog(s) of this gene implicated in cytochrome-c oxidase deficiency disease. Orthologous to human COA8 (cytochrome c oxidase assembly factor 8). [provided by Alliance of Genome Resources, Apr 2022]