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 [Source:SGD;Acc:S000001795]

Chromosome XI: 603,195-604,232 reverse strand.

R64-1-1:BK006944.2

This gene has 1 transcript (splice variant), 219 orthologues and is a member of 2 Ensembl protein families.

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