Mitofilin complexes: conserved organizers of mitochondrial membrane architecture

Biol Chem. 2012 Nov;393(11):1247-61. doi: 10.1515/hsz-2012-0239.

Abstract

Mitofilin proteins are crucial organizers of mitochondrial architecture. They are located in the inner mitochondrial membrane and interact with several protein complexes of the outer membrane, thereby generating contact sites between the two membrane systems of mitochondria. Within the inner membrane, mitofilins are part of hetero-oligomeric protein complexes that have been termed the mitochondrial inner membrane organizing system (MINOS). MINOS integrity is required for the maintenance of the characteristic morphology of the inner mitochondrial membrane, with an inner boundary region closely apposed to the outer membrane and cristae membranes, which form large tubular invaginations that protrude into the mitochondrial matrix and harbor the enzyme complexes of the oxidative phosphorylation machinery. MINOS deficiency comes along with a loss of crista junction structures and the detachment of cristae from the inner boundary membrane. MINOS has been conserved in evolution from unicellular eukaryotes to humans, where alterations of MINOS subunits are associated with multiple pathological conditions.

Publication types

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

MeSH terms

  • Animals
  • Conserved Sequence*
  • Humans
  • Mitochondrial Membranes / chemistry
  • Mitochondrial Membranes / metabolism*
  • Mitochondrial Proteins / chemistry*
  • Mitochondrial Proteins / metabolism*
  • Models, Biological
  • Muscle Proteins / chemistry
  • Muscle Proteins / metabolism

Substances

  • IMMT protein, human
  • Mitochondrial Proteins
  • Muscle Proteins