Pathophysiology of mitochondrial volume homeostasis: potassium transport and permeability transition

Biochim Biophys Acta. 2009 May;1787(5):345-50. doi: 10.1016/j.bbabio.2008.10.006. Epub 2008 Oct 25.


Regulation of mitochondrial volume is a key issue in cellular pathophysiology. Mitochondrial volume and shape changes can occur following regulated fission-fusion events, which are modulated by a complex network of cytosolic and mitochondrial proteins; and through regulation of ion transport across the inner membrane. In this review we will cover mitochondrial volume homeostasis that depends on (i) monovalent cation transport across the inner membrane, a regulated process that couples electrophoretic K(+) influx on K(+) channels to K(+) extrusion through the K(+)-H(+) exchanger; (ii) the permeability transition, a loss of inner membrane permeability that may be instrumental in triggering cell death. Specific emphasis will be placed on molecular advances on the nature of the transport protein(s) involved, and/or on diseases that depend on mitochondrial volume dysregulation.

Publication types

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

MeSH terms

  • Biological Transport
  • Cell Membrane Permeability / physiology
  • Homeostasis
  • Humans
  • Mitochondria / physiology*
  • Mitochondria / ultrastructure*
  • Mitochondrial Swelling
  • Muscular Dystrophies / physiopathology
  • Permeability
  • Potassium / metabolism*
  • Potassium Channels / physiology*
  • Potassium-Hydrogen Antiporters / physiology*
  • Reactive Oxygen Species / metabolism
  • Wolf-Hirschhorn Syndrome / physiopathology


  • Potassium Channels
  • Potassium-Hydrogen Antiporters
  • Reactive Oxygen Species
  • Potassium