The mitochondria are double-membrane organelles integral for energy metabolism. Mitochondrial dynamics is regulated by inner and outer mitochondrial membrane (IMM and OMM) proteins, which promote fission and fusion. Optic atrophy 1 (OPA1) regulates IMM fusion, prevents apoptosis, and is a key regulator of morphological change in skeletal and cardiac muscle physiology and pathophysiology. OPA1 fuses the inner membranes of adjacent mitochondria, allowing for an increase in oxidative phosphorylation (OXPHOS). Considering the importance of energy metabolism in whole-body physiology, OPA1 and its regulators have been proposed as novel targets for the treatment of skeletal muscle atrophy and heart failure. Here, we review the role and regulation of OPA1 in skeletal muscle and cardiac pathophysiology, epitomizing its critical role in the cell.

线粒体是能量代谢不可或缺的双膜细胞器。线粒体动力学由线粒体内外膜（IMM 和 OMM）蛋白调节，促进裂变和融合。视神经萎缩 1 (OPA1) 调节 IMM 融合，防止细胞凋亡，是骨骼和心肌生理学和病理生理学形态变化的关键调节因子。OPA1 融合相邻线粒体的内膜，从而增加氧化磷酸化 (OXPHOS)。考虑到能量代谢在全身生理学中的重要性，OPA1 及其调节剂已被提议作为治疗骨骼肌萎缩和心力衰竭的新靶点。在这里，我们回顾了 OPA1 在骨骼肌和心脏病理生理学中的作用和调节，体现了它在细胞中的关键作用。