Zhang Yifan, Bao Xingfu, Ma Jun, Lv Linhe, Hu Min, Wei Xiaoxi
Department of Orthodontics, Hospital of Stomatology, Jilin University, Changchun, China.
Department of Dentistry, Orthodontics and Craniofacial Biology, Research Institute for Medical Innovation, Radboud University Medical Center, Nijmegen, the Netherlands.
FASEB J. 2025 Jul 31;39(14):e70852. doi: 10.1096/fj.202501073RR.
Retinoic acid receptor-related orphan receptor beta (RORβ) is a ligand-dependent transcription factor essential for bone metabolism. While RORβ negatively regulates osteoblast differentiation and contributes to age-related or postmenopausal osteoporosis, its role in early skeletal development remains unclear. This study developed a Rorβ gene knockout (KO) mouse model using CRISPR/Cas9 to investigate its effects on endochondral ossification. At 4 weeks, Rorβ KO mice exhibited dwarfism and early-onset osteoporosis, with reduced femur length (-8.84%), lower cortical and trabecular bone mass, and impaired bone quality. The trabecular bone was fragile, with reduced surface osteoblasts and impaired osteogenic differentiation of bone marrow mesenchymal stem cells (BMSCs). Chondrogenesis was also disrupted, evidenced by a thinner growth plate, fewer chondrocytes, and a disorganized hypertrophic zone (HZ). The expressions of proliferation (Ki67) and hypertrophic differentiation (Collagen X) markers were significantly reduced. Mechanistically, RORβ deficiency downregulated hypoxia-inducible factor 1-alpha (HIF-1α) and its downstream target vascular endothelial growth factor A (VEGFA) in both the pre-hypertrophic zone (PHZ) of the growth plate and BMSCs. These findings identify RORβ as a critical regulator of endochondral ossification, linking its loss to skeletal defects via impaired HIF-1α/VEGFA signaling. This study provides insights into potential therapeutic strategies for skeletal diseases such as skeletal dysplasia.
维甲酸受体相关孤儿受体β(RORβ)是一种对骨代谢至关重要的配体依赖性转录因子。虽然RORβ对成骨细胞分化起负调节作用,并导致与年龄相关或绝经后骨质疏松症,但它在早期骨骼发育中的作用仍不清楚。本研究使用CRISPR/Cas9技术构建了Rorβ基因敲除(KO)小鼠模型,以研究其对软骨内成骨的影响。4周龄时,Rorβ基因敲除小鼠表现出侏儒症和早发性骨质疏松症,股骨长度缩短(-8.84%),皮质骨和小梁骨质量降低,骨质量受损。小梁骨脆弱,表面成骨细胞减少,骨髓间充质干细胞(BMSC)的成骨分化受损。软骨形成也受到破坏,表现为生长板变薄、软骨细胞减少以及肥大带(HZ)紊乱。增殖(Ki67)和肥大分化(胶原蛋白X)标志物的表达显著降低。机制上,RORβ缺乏下调了生长板的前肥大区(PHZ)和骨髓间充质干细胞中缺氧诱导因子1α(HIF-1α)及其下游靶标血管内皮生长因子A(VEGFA)。这些发现确定RORβ是软骨内成骨的关键调节因子,通过受损的HIF-1α/VEGFA信号通路将其缺失与骨骼缺陷联系起来。本研究为骨骼发育异常等骨骼疾病的潜在治疗策略提供了见解。