
Mitochondria amplify activation of caspases during apoptosis by releasing cytochrome c and other cofactors. This is accompanied by fragmentation of the organelle and remodeling of the cristae. Here we provide evidence that Optic Atrophy 1 (OPA1), a profusion dynamin-related protein of the inner mitochondrial membrane mutated in dominant optic atrophy, protects from apoptosis by preventing cytochrome c release independently from mitochondrial fusion. OPA1 does not interfere with activation of the mitochondrial "gatekeepers" BAX and BAK, but it controls the shape of mitochondrial cristae, keeping their junctions tight during apoptosis. Tightness of cristae junctions correlates with oligomerization of two forms of OPA1, a soluble, intermembrane space and an integral inner membrane one. The proapoptotic BCL-2 family member BID, which widens cristae junctions, also disrupts OPA1 oligomers. Thus, OPA1 has genetically and molecularly distinct functions in mitochondrial fusion and in cristae remodeling during apoptosis.
Cachexia, PROTEIN, Apoptosis, Membrane Fusion, GTP Phosphohydrolases, Mice, MEDIATOR, Lymphocytes, Cells, Cultured, 11 Medical and Health Sciences, REQUIRES, bcl-2-Associated X Protein, Mice, Knockout, Research Support, Non-U.S. Gov't, Cytochromes c, FISSION, Mitochondria, bcl-2 Homologous Antagonist-Killer Protein, Proto-Oncogene Proteins c-bcl-2, Mitochondrial Membranes, Female, Life Sciences & Biomedicine, Signal Transduction, Biochemistry & Molecular Biology, CYTOCHROME-C RELEASE, 610, Down-Regulation, Genetics and Molecular Biology, Cell Line, Tight Junctions, Mitochondrial Proteins, Animals, MITOFUSIN-1, Science & Technology, Biochemistry, Genetics and Molecular Biology(all), 31 Biological sciences, Cell Biology, 32 Biomedical and clinical sciences, 06 Biological Sciences, Mice, Inbred C57BL, CELL-DEATH, BAX, General Biochemistry, Metalloproteases, MORPHOLOGY, 060112 Structural Biology (incl. Macromolecular Modelling), Genes, Lethal, GTPASE, Developmental Biology
Cachexia, PROTEIN, Apoptosis, Membrane Fusion, GTP Phosphohydrolases, Mice, MEDIATOR, Lymphocytes, Cells, Cultured, 11 Medical and Health Sciences, REQUIRES, bcl-2-Associated X Protein, Mice, Knockout, Research Support, Non-U.S. Gov't, Cytochromes c, FISSION, Mitochondria, bcl-2 Homologous Antagonist-Killer Protein, Proto-Oncogene Proteins c-bcl-2, Mitochondrial Membranes, Female, Life Sciences & Biomedicine, Signal Transduction, Biochemistry & Molecular Biology, CYTOCHROME-C RELEASE, 610, Down-Regulation, Genetics and Molecular Biology, Cell Line, Tight Junctions, Mitochondrial Proteins, Animals, MITOFUSIN-1, Science & Technology, Biochemistry, Genetics and Molecular Biology(all), 31 Biological sciences, Cell Biology, 32 Biomedical and clinical sciences, 06 Biological Sciences, Mice, Inbred C57BL, CELL-DEATH, BAX, General Biochemistry, Metalloproteases, MORPHOLOGY, 060112 Structural Biology (incl. Macromolecular Modelling), Genes, Lethal, GTPASE, Developmental Biology
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