Metalloendopeptidase of the mitochondrial inner membrane; important for adaptive responses to various homeostatic insults, preservation of normal mitochondrial function under damage-eliciting conditions, and stability of respiratory supercomplexes; involved in turnover of membrane-embedded proteins; mediates degradation of Cox1p in coa2 mutant cells; zebrafish ortholog has a role in organ development and mouse ortholog is also required for respiratory supercomplex stability
