Erlebacher A, Derynck R
Department of Growth and Development, University of California at San Francisco 94143, USA.
J Cell Biol. 1996 Jan;132(1-2):195-210. doi: 10.1083/jcb.132.1.195.
The development of the skeleton requires the coordinated activities of bone-forming osteoblasts and bone-resorbing osteoclasts. The activities of these two cell types are likely to be regulated by TGF-beta, which is abundant in bone matrix. We have used transgenic mice to evaluate the role of TGF-beta 2 in bone development and turnover. Osteoblast-specific overexpression of TGF-beta 2 from the osteocalcin promoter resulted in progressive bone loss associated with increases in osteoblastic matrix deposition and osteoclastic bone resorption. This phenotype closely resembles the bone abnormalities seen in human hyperparathyroidism and osteoporosis. Furthermore, a high level of TGF-beta 2 overexpression resulted in defective bone mineralization and severe hypoplasia of the clavicles, a hallmark of the developmental disease cleidocranial dysplasia. Our results suggest that TGF-beta 2 functions as a local positive regulator of bone remodeling and that alterations in TGF-beta 2 synthesis by bone cells, or in their responsiveness to TGF-beta 2, may contribute to the pathogenesis of metabolic bone disease.
骨骼的发育需要成骨的成骨细胞和吸收骨的破骨细胞的协同活动。这两种细胞类型的活动可能受转化生长因子-β(TGF-β)调节,TGF-β在骨基质中含量丰富。我们利用转基因小鼠评估了TGF-β2在骨骼发育和骨转换中的作用。从骨钙素启动子成骨细胞特异性过表达TGF-β2导致与成骨细胞基质沉积增加和破骨细胞骨吸收增加相关的进行性骨质流失。这种表型与人类甲状旁腺功能亢进和骨质疏松症中所见的骨骼异常非常相似。此外,高水平的TGF-β2过表达导致骨矿化缺陷和锁骨严重发育不全,这是发育性疾病锁骨颅骨发育不全的一个标志。我们的结果表明,TGF-β2作为骨重塑的局部正调节因子发挥作用,并且骨细胞TGF-β2合成的改变或它们对TGF-β2的反应性改变可能有助于代谢性骨病的发病机制。