title: Mitochondrial dynamics and disease, OPA1.
abstract: The mitochondria are dynamic organelles that constantly fuse and divide. An equilibrium between fusion and fission controls the morphology of the mitochondria, which appear as dots or elongated tubules depending the prevailing force. Characterization of the components of the fission and fusion machineries has progressed considerably, and the emerging question now is what role mitochondrial dynamics play in mitochondrial and cellular functions. Its importance has been highlighted by the discovery that two human diseases are caused by mutations in the two mitochondrial pro-fusion genes, MFN2 and OPA1. This review will focus on data concerning the function of OPA1, mutations in which cause optic atrophy, with respect to the underlying pathophysiological processes.
authors.affiliation: Laboratoire de Biologie Cellulaire et Moléculaire du Contrôle de la Prolifération, Université Paul Sabatier, 118 route de Narbonne, 31062 Toulouse, France.
authors.forename: Aurélien
authors.initials: A
authors.lastname: Olichon
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Guillou
authors.forename: Cécile
authors.initials: C
authors.lastname: Delettre
authors.forename: Thomas
authors.initials: T
authors.lastname: Landes
authors.forename: Laetitia
authors.initials: L
authors.lastname: Arnauné-Pelloquin
authors.forename: Laurent J
authors.initials: LJ
authors.lastname: Emorine
authors.forename: Valérie
authors.initials: V
authors.lastname: Mils
authors.forename: Marlène
authors.initials: M
authors.lastname: Daloyau
authors.forename: Christian
authors.initials: C
authors.lastname: Hamel
authors.forename: Patrizia
authors.initials: P
authors.lastname: Amati-Bonneau
authors.forename: Dominique
authors.initials: D
authors.lastname: Bonneau
authors.forename: Pascal
authors.initials: P
authors.lastname: Reynier
authors.forename: Guy
authors.initials: G
authors.lastname: Lenaers
authors.forename: Pascale
authors.initials: P
authors.lastname: Belenguer
mesh_terms: D020558:GTP Phosphohydrolases / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D019204:GTP-Binding Proteins / Q000378:metabolism; D006801:Humans; D008928:Mitochondria / Q000378:metabolism* / Q000473:pathology*; D024101:Mitochondrial Proteins / Q000378:metabolism; D009154:Mutation / Q000235:genetics; D029241:Optic Atrophy, Autosomal Dominant / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D029701:Saccharomyces cerevisiae Proteins / Q000378:metabolism; D015003:Yeasts / Q000378:metabolism
chemical_list: C082883:MGM1 protein, S cerevisiae; D024101:Mitochondrial Proteins; D029701:Saccharomyces cerevisiae Proteins; D020558:GTP Phosphohydrolases; D019204:GTP-Binding Proteins; C415198:OPA1 protein, human