![]() We demonstrate that OPA1 variants are synthesized with a bipartite-type mitochondrial targeting sequence. In yeast, mitochondrial rhomboid protease regulates mitochondrial function and morphology through proteolytic cleavage of Mgm1, the yeast homolog of OPA1. It has several splice variants and even a single variant is found as several processed forms, although their functional significance is unknown. The dynamin-like GTPase OPA1, a causal gene product of human dominant optic atrophy, functions in mitochondrial fusion and inner membrane remodeling. ![]()
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