Wang Yongmei, Nishida Shigeki, Sakata Takeshi, Elalieh Hashem Z, Chang Wenhan, Halloran Bernard P, Doty Steven B, Bikle Daniel D
Department of Medicine, Endocrine Unit, 111N, Veterans Affairs Medical Center, 4150 Clement Street, San Francisco, California 94121, USA.
Endocrinology. 2006 Oct;147(10):4753-61. doi: 10.1210/en.2006-0196. Epub 2006 Jul 20.
Although IGF-I has been identified as an important growth factor for the skeleton, the role of IGF-I on embryonic bone development remains unknown. Here we show that, in IGF-I-deficient (IGF-I(-/-)) mice, skeletal malformations, including short-limbed dwarfism, were evident at days post coitus (dpc) 14.5 to 18.5, accompanied by delays of mineralization in the spinal column, sternum, and fore paws. Reduced chondrocyte proliferation and increased chondrocyte apoptosis were identified in both the spinal ossification center and the growth plate of long bones. Abnormal chondrocyte differentiation and delayed initiation of mineralization was characterized by small size and fewer numbers of type X collagen expressing hypertrophic chondrocytes and lower osteocalcin expression. The Indian hedgehog-PTHrP feedback loop was altered; expression of Indian hedgehog was reduced in IGF-I(-/-) mice in long bones and in the spine, whereas expression of PTHrP was increased. Our results indicate that IGF-I plays an important role in skeletal development by promoting chondrocyte proliferation and maturation while inhibiting apoptosis to form bones of appropriate size and strength.
尽管胰岛素样生长因子-I(IGF-I)已被确定为骨骼的重要生长因子,但其在胚胎骨骼发育中的作用仍不清楚。在此我们表明,在IGF-I基因敲除(IGF-I(-/-))小鼠中,在交配后第14.5至18.5天出现明显的骨骼畸形,包括短肢侏儒症,同时伴有脊柱、胸骨和前爪矿化延迟。在脊柱骨化中心和长骨生长板中均发现软骨细胞增殖减少和软骨细胞凋亡增加。异常的软骨细胞分化和矿化起始延迟表现为表达X型胶原蛋白的肥大软骨细胞数量少且体积小,以及骨钙素表达降低。印度刺猬蛋白-甲状旁腺激素相关蛋白(Indian hedgehog-PTHrP)反馈回路发生改变;在IGF-I(-/-)小鼠的长骨和脊柱中,印度刺猬蛋白的表达降低,而PTHrP的表达增加。我们的结果表明,IGF-I通过促进软骨细胞增殖和成熟,同时抑制凋亡以形成大小和强度合适的骨骼,在骨骼发育中发挥重要作用。