Recombinant Human Mitochondrial-processing peptidase subunit alpha (PMPCA)

Uniprot Id

Q10713

Target Species

Human

Target Name

PMPCA

Target Full Name

Mitochondrial-processing peptidase subunit alpha

Target Function

Substrate recognition and binding subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins.

Target Involvement

Spinocerebellar ataxia, autosomal recessive, 2 (SCAR2)

Target Subcellular Location

Mitochondrion matrix. Mitochondrion inner membrane.

Target Protein Families

Peptidase M16 family

Target Tissue Specificity

Ubiquitously expressed with highest expression in fetal tissues and adult brain, cerebellum and cerebellar vermis.

Target Synonyms

1200002L24Rik; 4933435E07Rik; Alpha MPP; Alpha-MPP; FLJ26258; Inositol polyphosphate 5 phosphatase, 72 kD; INPP5E; KIAA0123; MGC104197; MGC93916; Mitochondrial matrix processing protease, alpha subunit; mitochondrial processing peptidase subunit alpha; Mitochondrial-processing peptidase subunit alpha; MPPA_HUMAN; P 55; P-55; Peptidase (mitochondrial processing) alpha; pmpca; RP11-413M3.1; RP23-306D20.8; SCAR2

Target Background

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.