The role of protein degradation in mitochondrial function and biogenesis

Curr Genet. 1996 Nov;30(5):367-80. doi: 10.1007/s002940050145.


It has been known for a long time that mitochondria contain their own protein-degradation systems. Only recently, however, have genes for mitochondrial proteases been identified and the powerful techniques of molecular biology been applied to gain insight into the role of protein degradation in mitochondrial biogenesis. It is now clear that the mitochondrial proteases that are involved in the initial stages of degradation are similar to prokaryotic ATP-dependent proteases, and that a division of labour exists between soluble and membrane-bound systems. These systems are essential for the biogenesis of fully functional mitochondria. Their natural targets are currently being identified, and their co-operation with chaperones and possible dual functions as chaperones/proteases are being investigated.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adenosine Triphosphate / metabolism
  • Endopeptidases / genetics
  • Endopeptidases / physiology*
  • Intracellular Membranes / chemistry
  • Intracellular Membranes / metabolism
  • Membrane Proteins / metabolism
  • Mitochondria / enzymology*
  • Mitochondria / genetics
  • Mitochondria / metabolism*
  • Models, Biological
  • Molecular Chaperones / physiology
  • Protein Biosynthesis
  • Proteins / metabolism*
  • Saccharomyces cerevisiae / enzymology
  • Saccharomyces cerevisiae / genetics


  • Membrane Proteins
  • Molecular Chaperones
  • Proteins
  • Adenosine Triphosphate
  • Endopeptidases