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Official Full Name: peptidase, mitochondrial processing subunit alpha
Also known as: CLA1; CPD3; MAS2; P-55; SCAR2; INPP5E; Alpha-MPP
The protein encoded by this gene is found in the mitochondrion, where it represents the alpha subunit of a proteolytic heterodimer. This heterodimer is responsible for cleaving the transit peptide from nuclear-encoded mitochondrial proteins. Defects in this gene are a cause of spinocerebellar ataxia, autosomal recessive 2.