Department of Cell Biology, State University of New York Downstate Medical Center,New York, New York, USA.
J Clin Invest. 2012 Nov;122(11):3873-87. doi: 10.1172/JCI62818.
The native capacity of adult skeletal muscles to regenerate is vital to the recovery from physical injuries and dystrophic diseases. Currently, the development of therapeutic interventions has been hindered by the complex regulatory network underlying the process of muscle regeneration. Using a mouse model of skeletal muscle regeneration after injury, we identified hexamethylene bisacetamide inducible 1 (HEXIM1, also referred to as CLP-1), the inhibitory component of the positive transcription elongation factor b (P-TEFb) complex, as a pivotal regulator of skeletal muscle regeneration. Hexim1-haplodeficient muscles exhibited greater mass and preserved function compared with those of WT muscles after injury, as a result of enhanced expansion of satellite cells. Transplanted Hexim1-haplodeficient satellite cells expanded and improved muscle regeneration more effectively than WT satellite cells. Conversely, HEXIM1 overexpression restrained satellite cell proliferation and impeded muscle regeneration. Mechanistically, dissociation of HEXIM1 from P-TEFb and subsequent activation of P-TEFb are required for satellite cell proliferation and the prevention of early myogenic differentiation. These findings suggest a crucial role for the HEXIM1/P-TEFb pathway in the regulation of satellite cell–mediated muscle regeneration and identify HEXIM1 as a potential therapeutic target for degenerative muscular diseases.
成年骨骼肌的固有再生能力对于从物理损伤和肌肉萎缩性疾病中恢复至关重要。目前,由于肌肉再生过程背后复杂的调控网络,治疗干预的发展受到了阻碍。我们使用一种损伤后骨骼肌再生的小鼠模型,鉴定出六亚甲基双乙酰胺诱导因子 1(HEXIM1,也称为 CLP-1),即正转录延伸因子 b(P-TEFb)复合物的抑制性成分,是骨骼肌再生的关键调节因子。Hexim1 单倍体缺乏的肌肉在损伤后表现出更大的质量和保留的功能,这是由于卫星细胞的扩增增强所致。移植的 Hexim1 单倍体缺乏的卫星细胞比 WT 卫星细胞更有效地扩增和改善肌肉再生。相反,HEXIM1 的过表达抑制了卫星细胞的增殖并阻碍了肌肉再生。从机制上讲,HEXIM1 从 P-TEFb 上解离以及随后 P-TEFb 的激活对于卫星细胞的增殖和早期成肌分化的预防是必需的。这些发现表明 HEXIM1/P-TEFb 途径在调节卫星细胞介导的肌肉再生中起着关键作用,并将 HEXIM1 鉴定为退行性肌肉疾病的潜在治疗靶点。