Mitochondrial processing peptidases

Oleksandr Gakh, Patrizia Cavadini, Grazia Isaya

Research output: Contribution to journalReview article

280 Scopus citations

Abstract

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
Volume1592
Issue number1
DOIs
StatePublished - Sep 2 2002

Keywords

  • Mitochondrion
  • Presequence
  • Processing peptidase
  • Protein import

ASJC Scopus subject areas

  • Molecular Biology
  • Cell Biology

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