Pmpca - peptidase, mitochondrial processing subunit alpha

Enables endopeptidase activity. Contributes to metallopeptidase activity. Involved in proteolysis. Predicted to be located in mitochondrial inner membrane. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in autosomal recessive spinocerebellar ataxia 2. Orthologous to human PMPCA (peptidase, mitochondrial processing subunit alpha). [provided by Alliance of Genome Resources, Apr 2022]