The oxidative phosphorylation (OXPHOS) system is intricately organized, with respiratory complexes forming super-assembled quaternary structures whose assembly mechanisms and physiological roles remain under investigation. Cox7a2l, also known as Scaf1, facilitates complex III and complex IV (CIII-CIV) super-assembly, enhancing energetic efficiency in various species. We examined the role of Cox7a1, another Cox7a family member, in supercomplex assembly and muscle physiology. Zebrafish lacking Cox7a1 exhibited reduced CIV₂ formation, metabolic alterations, and non-pathological muscle performance decline. Additionally, cox7a1^−/− hearts displayed a pro-regenerative metabolic profile, impacting cardiac regenerative response. The distinct phenotypic effects of cox7a1^−/− and cox7a2l^−/− underscore the diverse metabolic and physiological consequences of impaired supercomplex formation, emphasizing the significance of Cox7a1 in muscle maturation within the OXPHOS system.

氧化磷酸化（OXPHOS）系统组织复杂，呼吸复合物形成超组装四级结构，其组装机制和生理作用仍在研究中。 Cox7a2l，也称为 Scaf1，促进复合物 III 和复合物 IV (CIII-CIV) 超级组装，提高各种物种的能量效率。我们研究了 Cox7a1（另一个 Cox7a 家族成员）在超复杂组装和肌肉生理学中的作用。缺乏 Cox7a1 的斑马鱼表现出 CIV ₂形成减少、代谢改变和非病理性肌肉性能下降。此外，cox7a1 ^−/−心脏表现出促再生代谢特征，影响心脏再生反应。cox7a1 ^−/−和cox7a2l ^−/−的独特表型效应强调了超复合物形成受损的不同代谢和生理后果，强调了 Cox7a1 在 OXPHOS 系统内肌肉成熟中的重要性。