PMPCA( Human )
peptidase, mitochondrial processing alpha subunit
Alias
Alpha-MPP|INPP5E|P-55|SCAR2
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. [provided by RefSeq, Mar 2016].