The versatility of the mitochondrial presequence processing machinery: cleavage, quality control and turnover

Cell Tissue Res. 2017 Jan;367(1):73-81. doi: 10.1007/s00441-016-2492-9. Epub 2016 Sep 5.

Abstract

Mitochondria play a key role in several metabolic and cell biological pathways and have attracted increasing attention due to their implication in life-span, ageing and human diseases. Mitochondrial proteases have a special role in these multiple biological functions, as they are involved in the regulation of various processes, e.g., mitochondrial protein biogenesis and quality control, mitochondrial dynamics, mitophagy and programmed cell death. The mitochondrial presequence processing machinery serves the particular purpose of maturing the majority of incoming precursor proteins by presequence cleavage, to ensure a stable mature protein by trimming of intermediate N-termini and to remove free toxic targeting peptides.

Keywords: Mitochondria; Peptide turnover; Presequence proteases; Quality control; Targeting signals.

Publication types

  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Disease
  • Humans
  • Mitochondria / metabolism*
  • Mitochondrial Proteins / chemistry*
  • Mitochondrial Proteins / metabolism
  • Peptide Hydrolases / metabolism
  • Protein Processing, Post-Translational*

Substances

  • Mitochondrial Proteins
  • Peptide Hydrolases