Myoblast differentiation is a crucial process for myogenesis. Mitochondria function as an energy-providing machine that is critical to this process, and mitochondrial dysfunction can prevent myoblasts from fusing into myotubes. However, the molecular mechanisms underlying the dynamic regulation of mitochondrial networks remain poorly understood. In the present study, we found that the PTEN induced kinase 1(PINK1) /Parkin (an E3 ubiquitin-protein ligase) pathway is activated at the early stage of myoblast differentiation. Moreover, downregulation of mitofusin 2 (Mfn2) and increased dynamin-related protein 1 (Drp1) resulted in loosely formed mitochondria during this period. Furthermore, selective knockdown of the mitochondrial matrix protein Lon proteinase-1 (LonP1) at the early stage of myoblast differentiation induced mitochondrial depolarization and suppressed the PINK1/Parkin pathway and reduced Mfn2 and Drp1 levels, which blocked mitochondrial remodeling and myoblast differentiation. Overall, these data suggest that LonP1 plays an essential role in maintaining the normal myoblast differentiation process, which partly by regulating PINK1/Parkin mediated mitochondrial remodeling.

中文翻译：

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LonP1在成肌细胞分化过程中通过PINK1 / Parkin途径调节线粒体网络重塑。

成肌细胞分化是成肌的关键过程。线粒体作为能量供应的机器，对该过程至关重要，而线粒体功能障碍可阻止成肌细胞融合到肌管中。但是，线粒体网络的动态调节的分子机制仍然知之甚少。在本研究中，我们发现PTEN诱导的激酶1（PINK1）/ Parkin（E3泛素蛋白连接酶）途径在成肌细胞分化的早期被激活。此外，在此期间，线粒体蛋白2（Mfn2）的下调和动力蛋白相关蛋白1（Drp1）的增加导致线粒体形成松散。此外，在成肌细胞分化的早期选择性敲低线粒体基质蛋白Lon蛋白酶-1（LonP1）诱导线粒体去极化并抑制PINK1 / Parkin途径并降低Mfn2和Drp1水平，从而阻止了线粒体重塑和成肌细胞分化。总体而言，这些数据表明LonP1在维持正常的成肌细胞分化过程中起着至关重要的作用，部分是通过调节PINK1 / Parkin介导的线粒体重塑。