title: Mitochondrial fusion proteins: dual regulators of morphology and metabolism.
abstract: Mitochondria in mammalian cells are visualized as a network or as filaments that undergo continuous changes in shape and in localization within the cells. These changes are a consequence of the activity of different processes such as mitochondrial fusion and fission, and mitochondrial remodelling. In all, these processes are referred to as mitochondrial dynamics, and relevant questions, still unexplained, are why cells require such an active dynamics, or why mitochondria move to specific cellular regions. In this review we will summarize some of the biological functions assigned to the proteins identified as participating in mitochondrial fusion, namely mitofusin 1, mitofusin 2 and OPA1. In addition to the capacity of these proteins to promote fusion, mitofusin 2 or OPA1 regulate mitochondrial metabolism and loss-of-function reduces oxygen consumption and the capacity to oxidize substrates. We propose that mitochondrial fusion proteins operate as integrators of signals so they regulate both mitochondrial fusion and metabolism.
authors.affiliation: Institute for Research in Biomedicine (IRB Barcelona), Barcelona, Spain. antonio.zorzano@irbbarcelona.org
authors.forename: Antonio
authors.initials: A
authors.lastname: Zorzano
authors.forename: Marc
authors.initials: M
authors.lastname: Liesa
authors.forename: David
authors.initials: D
authors.lastname: Sebastián
authors.forename: Jessica
authors.initials: J
authors.lastname: Segalés
authors.forename: Manuel
authors.initials: M
authors.lastname: Palacín
mesh_terms: D000818:Animals; D020558:GTP Phosphohydrolases / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008561:Membrane Fusion / Q000502:physiology*; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism*; D026901:Membrane Transport Proteins / Q000235:genetics / Q000378:metabolism*; D008928:Mitochondria / Q000378:metabolism* / Q000648:ultrastructure*; D033681:Mitochondrial Membrane Transport Proteins; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: D008565:Membrane Proteins; D026901:Membrane Transport Proteins; D033681:Mitochondrial Membrane Transport Proteins; D024101:Mitochondrial Proteins; D020558:GTP Phosphohydrolases; C470018:MFN2 protein, human; C415198:OPA1 protein, human; C470017:Mfn1 protein, human