title: Pathophysiology of mitochondrial volume homeostasis: potassium transport and permeability transition.
abstract: 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.
authors.affiliation: Department of Genetics, University of Vienna, Austria.
authors.forename: Karin
authors.initials: K
authors.lastname: Nowikovsky
authors.forename: Rudolf J
authors.initials: RJ
authors.lastname: Schweyen
authors.forename: Paolo
authors.initials: P
authors.lastname: Bernardi
mesh_terms: D001692:Biological Transport; D002463:Cell Membrane Permeability / Q000502:physiology; D006706:Homeostasis; D006801:Humans; D008928:Mitochondria / Q000502:physiology* / Q000648:ultrastructure*; D008933:Mitochondrial Swelling; D009136:Muscular Dystrophies / Q000503:physiopathology; D010539:Permeability; D011188:Potassium / Q000378:metabolism*; D015221:Potassium Channels / Q000502:physiology*; D027961:Potassium-Hydrogen Antiporters / Q000502:physiology*; D017382:Reactive Oxygen Species / Q000378:metabolism; D054877:Wolf-Hirschhorn Syndrome / Q000503:physiopathology
chemical_list: D015221:Potassium Channels; D027961:Potassium-Hydrogen Antiporters; D017382:Reactive Oxygen Species; D011188:Potassium