The human dynamin-related protein OPA1 is anchored to the mitochondrial inner membrane facing the inter-membrane space.
Mutations in the OPA1 gene are associated with autosomal dominant optic atrophy. OPA1 encodes a dynamin-related protein orthologous to Msp1 of Schizosaccharomyces pombe and Mgm1p of Saccharomyces cerevisiae, both involved in mitochondrial morphology and genome maintenance. We present immuno-fluorescence and biochemical evidences showing that OPA1 resides in the mitochondria where it is imported through its highly basic amino-terminal extension. Proteolysis experiments indicate that OPA1 is present in the inter-membrane space and electron microscopy further localizes it close to the cristae. The strong association of OPA1 with membranes suggests its anchoring to the inner membrane.
Olichon
Aurélien
A
Laboratoire de Biologie Cellulaire et Moléculaire du Contrôle de la Prolifération and Laboratoire de Biologie Moléculaire des Eucaryotes, IFR109, Université Paul Sabatier, 118 route de Narbonne, 31062 cedex 04, Toulouse, France.
Emorine
Laurent J
LJ
Descoins
Eric
E
Pelloquin
Laetitia
L
Brichese
Laetitia
L
Gas
Nicole
N
Guillou
Emmanuelle
E
Delettre
Cécile
C
Valette
Annie
A
Hamel
Christian P
CP
Ducommun
Bernard
B
Lenaers
Guy
G
Belenguer
Pascale
P
D016475:3T3 Cells; D000818:Animals; D034281:Dynamins; D005455:Fluorescent Antibody Technique; D020558:GTP Phosphohydrolases / Q000378:metabolism*; D006367:HeLa Cells; D006801:Humans; D007425:Intracellular Membranes / Q000201:enzymology* / Q000378:metabolism; D051379:Mice; D008854:Microscopy, Electron; D008928:Mitochondria / Q000201:enzymology* / Q000378:metabolism; D051381:Rats
D020558:GTP Phosphohydrolases; C415198:OPA1 protein, human; C498809:Opa1 protein, mouse; C498811:Opa1 protein, rat; D034281:Dynamins