The human dynamin‐related protein OPA1 is anchored to the mitochondrial inner membrane facing the inter‐membrane space

FEBS Letters

Valette, Annie²  Descoins, Eric²  Emorine, Laurent J²  Pelloquin, Laetitia²  Delettre, Cécile¹  Brichese, Laetitia²  Gas, Nicole²  Guillou, Emmanuelle²  Olichon, Aurélien²  Ducommun, Bernard²  Hamel, Christian P¹  Lenaers, Guy²  Belenguer, Pascale² 

[1] INSERM U.254, Laboratoire de Neurobiologie de l'Audition, 71 rue de Navacelles, 34090 Montpellier, France;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 Toulouse cedex 04, France

关键词: Mitochondrion;    Dynamin;    Morphology;    Membrane;    Optic atrophy;    mts;    mitochondrial targeting signal;    ADOA;    autosomal dominant optic atrophy type 1;    DHFR;    dihydrofolate reductase;    IM;    inner membrane;    OM;    outer membrane;    IMS;    inter-membrane space;    GFP;    green fluorescent protein;   

DOI  :  10.1016/S0014-5793(02)02985-X

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

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.

【 授权许可】

Unknown