Njie Rohey, Xu Shihan, Wu Taofen, Pi Jiashun, Lin Sisi, Zhang Pengxiang, Wang Jiaqi, Dai Qi, Shen Hui, Zhang Nenghua, Chen Guiqian
Department of Biopharmaceutics, Zhejiang Provincial Engineering Research Center of New Technologies and Applications for Targeted Therapy of Major Diseases, College of Life Science and Medicine, Zhejiang Sci-Tech University, Hangzhou, China.
School of Life Sciences, University of Liverpool, Liverpool, UK.
J Cell Mol Med. 2025 Aug;29(16):e70813. doi: 10.1111/jcmm.70813.
The Hedgehog (Hh) signalling pathway serves as a fundamental regulator in bone development and homeostasis, translating extracellular signals into precise transcriptional programmes that govern osteogenic differentiation and bone remodelling. Central to this process, ligand-dependent Hh activation induces the nuclear translocation of GLI transcription factors (GLI1/2/3), which orchestrate the expression of key osteogenic regulators, including RUNX2 and Osterix (OSX), thereby directing mesenchymal stem cell (MSC) fate commitment. Among Hh ligands, the Indian hedgehog (Ihh) plays a dominant role in endochondral ossification, spatiotemporally controlling osteoprogenitor differentiation and chondrocyte maturation. Notably, the Hh pathway engages in extensive, context-dependent crosstalk with Wnt/β-catenin, BMP, TGF-β, FGF and PTHrP signalling cascades, forming a highly interconnected regulatory network essential for skeletal patterning and morphogenesis. Dysregulation of this balanced system contributes to a spectrum of skeletal disorders, ranging from congenital defects to degenerative bone diseases, highlighting its critical role in maintaining bone integrity. This review synthesises recent advances in Hh-mediated osteogenesis, dissecting its multi-layered interactions within the skeletal gene regulatory framework. By unravelling the molecular logic of Hh-dependent signalling networks, we deepen our understanding of bone biology and illuminate novel therapeutic targets for skeletal pathologies through precision modulation of Hh pathway activity.
刺猬(Hh)信号通路是骨骼发育和体内平衡的基本调节因子,它将细胞外信号转化为精确的转录程序,从而调控成骨细胞分化和骨骼重塑。在此过程中,配体依赖性Hh激活诱导GLI转录因子(GLI1/2/3)的核转位,这些转录因子协调关键成骨调节因子(包括RUNX2和osterix,OSX)的表达,从而指导间充质干细胞(MSC)的命运决定。在Hh配体中,印度刺猬因子(Ihh)在软骨内骨化中起主导作用,在时空上控制骨祖细胞分化和软骨细胞成熟。值得注意的是,Hh信号通路与Wnt/β-连环蛋白、BMP、TGF-β、FGF和PTHrP信号级联进行广泛的、依赖于环境的相互作用,形成一个对骨骼模式形成和形态发生至关重要的高度互联的调节网络。这个平衡系统的失调会导致一系列骨骼疾病,从先天性缺陷到退行性骨病,突出了其在维持骨骼完整性方面的关键作用。本综述总结了Hh介导的成骨作用的最新进展,剖析了其在骨骼基因调控框架内的多层相互作用。通过揭示Hh依赖性信号网络的分子逻辑,我们加深了对骨生物学的理解,并通过精确调节Hh信号通路活性为骨骼疾病阐明了新的治疗靶点。