Department of Neurology, David Geffen School of Medicine, University of California Los Angeles, 710 Westwood Plaza, Los Angeles, CA 90095, USA.
Hum Mol Genet. 2013 Aug 15;22(16):3283-95. doi: 10.1093/hmg/ddt184. Epub 2013 Apr 23.
Cell-mediated regenerative approaches using muscle progenitor cells hold promises for the treatment of many forms of muscle disorders. Their applicability in the clinic, however, is hindered by the low levels of regeneration obtained after transplantation and the large number of cells required to achieve an effect. To better understand the mechanisms that regulate the temporal switch of replicating muscle progenitor cells into terminally differentiated cells and to develop new strategies that could enhance muscle regeneration, we have developed and performed a high-throughput screening (HTS) capable of identifying genes that play active roles during myogenesis. Secondary and tertiary screens were used to confirm the effects of RNAi in vitro and in vivo and to select for candidate hits that significantly increase regeneration into skeletal muscles. Downregulation of cyclin D2 (CCND2) was shown to dramatically enhance myogenic differentiation of muscle progenitor cells and to induce a robust regeneration after cell transplantation into skeletal muscles of dystrophin-deficient mice. Protein interaction network and pathway analysis revealed that CCND2 directly interacts with the cyclin-dependent kinase Cdk4 to inhibit phosphorylation of the retinoblastoma protein (pRb), thus blocking the activation of the myogenic switch during fusion. These studies identify CCND2 as a new key regulator of terminal differentiation in muscle progenitor cells and open new possibilities for the treatment of many forms of muscle disorders characterized by impaired regeneration and loss of muscle mass.
利用肌肉祖细胞的细胞介导的再生方法有望治疗多种形式的肌肉疾病。然而,其在临床上的应用受到限制,因为移植后获得的再生水平较低,并且需要大量细胞才能产生效果。为了更好地了解调节复制性肌肉祖细胞向终末分化细胞的时间转换的机制,并开发可增强肌肉再生的新策略,我们开发并进行了高通量筛选(HTS),以鉴定在肌发生过程中发挥积极作用的基因。二级和三级筛选用于体外和体内确认 RNAi 的作用,并选择候选命中,这些命中可显著增加向骨骼肌的再生。下调细胞周期蛋白 D2(CCND2)可显著增强肌肉祖细胞的成肌分化,并在将细胞移植到肌营养不良症小鼠的骨骼肌后诱导强烈的再生。蛋白质相互作用网络和途径分析表明,CCND2 直接与细胞周期蛋白依赖性激酶 Cdk4 相互作用,抑制视网膜母细胞瘤蛋白(pRb)的磷酸化,从而阻止融合过程中肌生成开关的激活。这些研究将 CCND2 鉴定为肌肉祖细胞终末分化的新关键调节剂,并为许多以再生受损和肌肉量丧失为特征的肌肉疾病的治疗开辟了新的可能性。