Targeted disruption of Cbfa1 results in a complete lack of bone formation owing to maturational arrest of osteoblasts
Targeted disruption of Cbfa1 results in a complete lack of bone formation owing to maturational arrest of osteoblasts
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DOI：
10.1016/s0092-8674(00)80258-5
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发表时间：
1997-05-30
期刊：
影响因子：
64.5
通讯作者：
Kishimoto, T
中科院分区：
文献类型：
--
作者：
Komori, T;Yagi, H;Kishimoto, T
转录因子Cbfa1属于租赁结构域基因家族，在胎儿发育中限制性表达。为了阐明Cbfa1的功能，我们产生了突变的Cbfa1基因座的小鼠。具有Cbfa1纯合突变的小鼠在出生后立即死亡，没有呼吸。对他们骨骼系统的检查显示完全没有骨化。虽然未成熟的成骨细胞，表达碱性磷酸酶弱，但不骨桥蛋白和骨钙素，和一些未成熟的破骨细胞出现在软骨膜区域，既没有血管也没有间充质细胞入侵的软骨观察。因此，我们的数据表明，由于突变小鼠成骨细胞的成熟停滞，膜内和软骨内骨化都被完全阻断，并证明Cbfa1在骨生成中起着重要作用。
A transcription factor, Cbfa1, which belongs to the rent-domain gene family, is expressed restrictively in fetal development. To elucidate the function of Cbfa1, we generated mice with a mutated Cbfa1 locus. Mice with a homozygous mutation in Cbfa1 died just after birth without breathing. Examination of their skeletal systems showed a complete lack of ossification. Although immature osteoblasts, which expressed alkaline phophatase weakly but not Osteopontin and Osteocalcin, and a few immature osteoclasts appeared at the perichondrial region, neither vascular nor mesenchymal cell invasion was observed in the cartilage. Therefore, our data suggest that both intramembranous and endochondral ossification were completely blocked, owing to the maturational arrest of osteoblasts in the mutant mice, and demonstrate that Cbfa1 plays an essential role in osteogenesis.