Sprott Center for Stem Cell Research, Ottawa Hospital Research Institute, Regenerative Medicine Program, Ottawa, ON, Canada; Department of Cellular and Molecular Medicine, Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada.
Department of Cellular and Molecular Medicine, Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada.
Cell Stem Cell. 2019 Mar 7;24(3):419-432.e6. doi: 10.1016/j.stem.2019.01.002. Epub 2019 Jan 31.
Loss of dystrophin expression in Duchenne muscular dystrophy (DMD) causes progressive degeneration of skeletal muscle, which is exacerbated by reduced self-renewing asymmetric divisions of muscle satellite cells. This, in turn, affects the production of myogenic precursors and impairs regeneration and suggests that increasing such divisions may be beneficial. Here, through a small-molecule screen, we identified epidermal growth factor receptor (EGFR) and Aurora kinase A (Aurka) as regulators of asymmetric satellite cell divisions. Inhibiting EGFR causes a substantial shift from asymmetric to symmetric division modes, whereas EGF treatment increases asymmetric divisions. EGFR activation acts through Aurka to orient mitotic centrosomes, and inhibiting Aurka blocks EGF stimulation-induced asymmetric division. In vivo EGF treatment markedly activates asymmetric divisions of dystrophin-deficient satellite cells in mdx mice, increasing progenitor numbers, enhancing regeneration, and restoring muscle strength. Therefore, activating an EGFR-dependent polarity pathway promotes functional rescue of dystrophin-deficient satellite cells and enhances muscle force generation.
肌营养不良症(DMD)中肌营养不良蛋白的表达缺失导致骨骼肌进行性退化,而肌肉卫星细胞自我更新的不对称分裂减少会加剧这种退化。这反过来又会影响肌原性前体细胞的产生,并损害再生,这表明增加此类分裂可能是有益的。在这里,我们通过一个小分子筛选发现表皮生长因子受体(EGFR)和 Aurora 激酶 A(Aurka)是不对称卫星细胞分裂的调节因子。抑制 EGFR 会导致不对称分裂模式向对称分裂模式的显著转变,而 EGF 处理则增加不对称分裂。EGFR 激活通过 Aurka 来定向有丝分裂中心体,而抑制 Aurka 则阻断 EGF 刺激诱导的不对称分裂。体内 EGF 处理显著激活 mdx 小鼠中缺失 dystrophin 的卫星细胞的不对称分裂,增加祖细胞数量,增强再生,并恢复肌肉力量。因此,激活依赖于 EGFR 的极性途径可促进功能缺失的肌营养不良蛋白卫星细胞的恢复,并增强肌肉力量的产生。