Mitochondrial processing peptidases

Oleksandr Gakh, Patrizia Cavadini, Grazia Isaya

Research output: Contribution to journalReview articlepeer-review

298 Scopus citations


Three peptidases are responsible for the proteolytic processing of both nuclearly and mitochondrially encoded precursor polypeptides targeted to the various subcompartments of the mitochondria. Mitochondrial processing peptidase (MPP) cleaves the vast majority of mitochondrial proteins, while inner membrane peptidase (IMP) and mitochondrial intermediate peptidase (MIP) process specific subsets of precursor polypeptides. All three enzymes are structurally and functionally conserved across species, and their human homologues begin to be recognized as potential players in mitochondrial disease.

Original languageEnglish (US)
Pages (from-to)63-77
Number of pages15
JournalBiochimica et Biophysica Acta - Molecular Cell Research
Issue number1
StatePublished - Sep 2 2002


  • Mitochondrion
  • Presequence
  • Processing peptidase
  • Protein import

ASJC Scopus subject areas

  • Molecular Biology
  • Cell Biology


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