PMPCA Substrate recognition and binding subunit of the essential mitochondrial processing protease (MPP), which is required for maturation of the majority of mitochondrial precursor proteins. Most MPP cleavage sites follow an arginine at position -2. Belongs to the peptidase M16 family. Ubiquitously expressed with highest expression in fetal tissues and adult brain, cerebellum and cerebellar vermis. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC; Mitochondrial; Protease
Chromosomal Location of human Ortholog: 9q34.3
Cellular Component:  mitochondrial inner membrane; mitochondrial processing peptidase complex; mitochondrion
Molecular Function:  metal ion binding; metalloendopeptidase activity; protein binding
Biological Process:  protein processing involved in protein targeting to mitochondrion
Disease: Spinocerebellar Ataxia, Autosomal Recessive 2
Reference #:  Q10713 (UniProtKB)
Alt. Names/Synonyms: Alpha-MPP; cerebellar ataxia 1 (autosomal recessive); Cerebellar ataxia-1; CLA1; CPD3; FLJ26258; Inactive zinc metalloprotease alpha; inositol polyphosphate-5-phosphatase, 72 kD; INPP5E; KIAA0123; MAS2; MGC104197; mitochondrial matrix processing protease, alpha subunit; Mitochondrial-processing peptidase subunit alpha; MPPA; P-55; peptidase (mitochondrial processing) alpha; peptidase, mitochondrial processing alpha subunit; peptidase, mitochondrial processing subunit alpha; PMPCA; SCAR2
Gene Symbols: PMPCA
Molecular weight: 58,253 Da
Basal Isoelectric point: 6.45  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein