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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.

Original publication

DOI

10.1016/j.semcdb.2009.12.012

Type

Journal article

Journal

Semin Cell Dev Biol

Publication Date

08/2010

Volume

21

Pages

593 - 598

Keywords

Animals, Apoptosis, DNA, Mitochondrial, Energy Metabolism, GTP Phosphohydrolases, Humans, Membrane Fusion, Mitochondria, Mitochondrial Membranes, Mitochondrial Proteins, Mutation, Optic Atrophy, Autosomal Dominant