OPA1 (optic atrophy protein 1) is a dynamin-related GTPase which forms a diffusion barrier for proteins stored in mitochondrial cristae and is critical to mitochondrial fusion as well as regulation of apoptosis. Intrinsic apoptotic signals mediated proteolytic processing leads OPA1 oligomers disassembly and caspase activator cytochrome C (CYCS) release into mitochondrial intermembrane spaces. Defects in OPA1 are a cause of optic atrophy type 1 (OPA1) as well as dominant optic atrophy plus syndrome (DOA+).
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