title: OPA1 (dys)functions.
abstract: Mitochondrial morphology varies according to cell type and cellular context from an interconnected filamentous network to isolated dots. This morphological plasticity depends on mitochondrial dynamics, a balance between antagonistic forces of fission and fusion. DRP1 and FIS1 control mitochondrial outer membrane fission and Mitofusins its fusion. This review focuses on OPA1, one of the few known actors of inner membrane dynamics, whose mutations provoke an optic neuropathy. Since its first identification in 2000 the characterization of the functions of OPA1 has made rapid progress thus providing numerous clues to unravel the pathogenetic mechanisms of ADOA-1.
authors.affiliation: Université de Toulouse, UPS, CNRS, UMR 52412, Laboratoire Métabolisme Plasticité Mitochondries, Toulouse, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Landes
authors.forename: Ingrid
authors.initials: I
authors.lastname: Leroy
authors.forename: Ambre
authors.initials: A
authors.lastname: Bertholet
authors.forename: Alan
authors.initials: A
authors.lastname: Diot
authors.forename: Farnoosh
authors.initials: F
authors.lastname: Khosrobakhsh
authors.forename: Marlène
authors.initials: M
authors.lastname: Daloyau
authors.forename: Noélie
authors.initials: N
authors.lastname: Davezac
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Miquel
authors.forename: Delphine
authors.initials: D
authors.lastname: Courilleau
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Guillou
authors.forename: Aurélien
authors.initials: A
authors.lastname: Olichon
authors.forename: Guy
authors.initials: G
authors.lastname: Lenaers
authors.forename: Laetitia
authors.initials: L
authors.lastname: Arnauné-Pelloquin
authors.forename: Laurent J
authors.initials: LJ
authors.lastname: Emorine
authors.forename: Pascale
authors.initials: P
authors.lastname: Belenguer
mesh_terms: D000818:Animals; D017209:Apoptosis; D004272:DNA, Mitochondrial / Q000378:metabolism; D004734:Energy Metabolism; D020558:GTP Phosphohydrolases / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008561:Membrane Fusion; D008928:Mitochondria / Q000378:metabolism* / Q000648:ultrastructure; D051336:Mitochondrial Membranes / Q000378:metabolism / Q000648:ultrastructure; D024101:Mitochondrial Proteins / Q000378:metabolism*; D009154:Mutation; D029241:Optic Atrophy, Autosomal Dominant / Q000503:physiopathology
chemical_list: D004272:DNA, Mitochondrial; D024101:Mitochondrial Proteins; D020558:GTP Phosphohydrolases; C415198:OPA1 protein, human