EC - Mitochondrial processing peptidase

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IntEnz Enzyme Nomenclature


Accepted name:
mitochondrial processing peptidase
Other names:
matrix peptidase
matrix processing peptidase
matrix processing proteinase
mitochondrial protein precursor-processing proteinase
processing enhancing peptidase (for one of two subunits)
processing enhancing peptidase
Systematic name:




Known from the mitochondrial matrix of fungi and mammals. Formed from two subunits, both homologous with pitrilysin [3], and the products of the MAS1 and MAS2 genes in yeast. In peptidase family M16 (pitrilysin family). Formerly EC

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , MEROPS , UniPathway
Protein domains and families: PROSITE:PDOC00130
Structural data: CSA , EC2PDB
Gene Ontology: GO:0004240
CAS Registry Number: 86280-61-7
UniProtKB/Swiss-Prot: (24) [show] [UniProt]


  1. Jensen, R.E. and Yaffe, M.P.
    Import of proteins into yeast mitochondria: the nuclear MAS2 gene encodes a component of the processing protease that is homologous to the MAS1-encoded subunit.
    EMBO J. 7: 3863-3871 (1988). [PMID: 3061808]
  2. Witte, C., Jensen, R.E., Yaffe, M.P. and Schatz, G.
    MAS1, a gene essential for yeast mitochondrial assembly, encodes a subunit of the mitochondrial processing protease.
    EMBO J. 7: 1439-1447 (1988). [PMID: 3044780]
  3. Rawlings, N.D. and Barrett, A.J.
    Homologues of insulinase, a new superfamily of metalloendopeptidases.
    Biochem. J. 275: 389-391 (1991). [PMID: 2025223]
  4. Kalousek, F., Neupert, W., Omura, T., Schatz, G. and Schmitz, U.K.
    Uniform nomenclature for the mitochondrial peptidases cleaving precursors of mitochondrial proteins.
    Trends Biochem. Sci. 18: 249 (1993). [PMID: 8212133]
  5. Brunner, M. and Neupert, W.
    Purification and characterization of the mitochondrial processing peptidase of Neurospora crassa.
    Methods Enzymol. 248: 717-728 (1994).

[EC created 1989/90 as EC, transferred 1995 to EC]