Predicted to enable protein-disulfide reductase activity. Acts upstream of or within several processes, including copper ion homeostasis; respiratory chain complex IV assembly; and response to activity. Located in mitochondrion. Is expressed in several structures, including alimentary system; aorta; blastocyst; gonad; and hemolymphoid system gland. Used to study cytochrome-c oxidase deficiency disease. Human ortholog(s) of this gene implicated in cytochrome-c oxidase deficiency disease; fatal infantile cardioencephalomyopathy due to cytochrome c oxidase deficiency 1; hypertrophic cardiomyopathy; and myopia. Orthologous to human SCO2 (synthesis of cytochrome C oxidase 2). [provided by Alliance of Genome Resources, Apr 2022]