May act as a codon-independent translation release factor that has lost all stop codon specificity and directs the termination of translation in mitochondrion.
Involvement in disease
Defects in C12orf65 are the cause of combined oxidative phosphorylation deficiency type 7 (COXPD7) [MIM:613559]. A mitochondrial disease resulting in encephalomyopathy. Clinical manifestations include psychomotor delay and regression, ataxia, optic atrophy, nystagmus and muscle atrophy and weakness.
Belongs to the prokaryotic/mitochondrial release factor family.