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Olichon, Aurélien; Emorine, Laurent J; Descoins, Eric; Pelloquin, Laetitia; Brichese, Laetitia; Gas, Nicole; Guillou, Emmanuelle; Delettre, Cécile; Valette, Annie; Hamel, Christian P; Ducommun, Bernard; Lenaers, Guy; Belenguer, Pascale
FEBS letters, July 17, 2002, Letnik: 523, Številka: 1Journal Article
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.
