OPA1 (dys)functions.
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.
Landes
Thomas
T
Université de Toulouse, UPS, CNRS, UMR 52412, Laboratoire Métabolisme Plasticité Mitochondries, Toulouse, France.
Leroy
Ingrid
I
Bertholet
Ambre
A
Diot
Alan
A
Khosrobakhsh
Farnoosh
F
Daloyau
Marlène
M
Davezac
Noélie
N
Miquel
Marie-Christine
MC
Courilleau
Delphine
D
Guillou
Emmanuelle
E
Olichon
Aurélien
A
Lenaers
Guy
G
Arnauné-Pelloquin
Laetitia
L
Emorine
Laurent J
LJ
Belenguer
Pascale
P
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
D004272:DNA, Mitochondrial; D024101:Mitochondrial Proteins; D020558:GTP Phosphohydrolases; C415198:OPA1 protein, human