3U Diabetes Partnership, School of Health and Human Performance, Dublin City University, Dublin, Ireland; National Institute for Cellular Biotechnology, Dublin City University, Dublin, Ireland; School of Biochemistry and Immunology, Trinity Biomedical Sciences Institute (TBSI), Trinity College Dublin, Dublin, Ireland; Translational Research Institute, AdventHealth, Orlando, FL, USA.
3U Diabetes Partnership, School of Health and Human Performance, Dublin City University, Dublin, Ireland; National Institute for Cellular Biotechnology, Dublin City University, Dublin, Ireland; SSPC, The SFI Research Centre for Pharmaceuticals, Bernal Institute, University of Limerick, Limerick, Ireland.
Trends Endocrinol Metab. 2022 Oct;33(10):710-721. doi: 10.1016/j.tem.2022.07.003. Epub 2022 Aug 6.
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.
线粒体是双层膜细胞器，对能量代谢至关重要。线粒体动力学受内外膜（IMM 和 OMM）蛋白的调节，这些蛋白促进分裂和融合。视神经萎缩 1（OPA1）调节 IMM 融合，防止细胞凋亡，是调节骨骼肌和心肌生理学和病理生理学形态变化的关键调节剂。OPA1 融合相邻线粒体的内膜，增加氧化磷酸化（OXPHOS）。鉴于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，我们综述了 OPA1 在骨骼肌和心脏病理生理学中的作用和调节，强调了它在细胞中的关键作用。
