A MURINE SKELETAL ADAPTATION THAT SIGNIFICANTLY INCREASES CORTICAL BONE MECHANICAL-PROPERTIES - IMPLICATIONS FOR HUMAN SKELETAL FRAGILITY
A MURINE SKELETAL ADAPTATION THAT SIGNIFICANTLY INCREASES CORTICAL BONE MECHANICAL-PROPERTIES - IMPLICATIONS FOR HUMAN SKELETAL FRAGILITY
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DOI：
10.1172/jci116756
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发表时间：
1993-10-01
影响因子：
15.9
通讯作者：
GOLDSTEIN, SA
中科院分区：
文献类型：
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作者：
BONADIO, J;JEPSEN, KJ;GOLDSTEIN, SA
Mov13小鼠携带一种前病毒，可阻止α 1（I）胶原基因的转录起始。无效突变的纯合子突变小鼠不产生I型胶原蛋白，并在妊娠中期死亡，而杂合子则存活至成年。来自杂合小鼠的真皮成纤维细胞产生的I型胶原比正常同窝小鼠少约50%，并且胶原产生的部分缺陷导致类似于I型成骨细胞（骨骼脆性的遗传形式）的表型。在这项研究中，我们已经确定了一个适应Mov13骨骼组织，显着提高长骨的弯曲强度。适应性反应发生在2个月的时间，在此期间，少量的新增殖的成骨细胞产生大量的基质成分，从而产生新的骨沿着骨膜表面。新骨沉积导致横截面几何形状显著增加，进而导致长骨弯曲强度显著增加。
Mov13 mice carry a provirus that prevents transcription initiation of the alpha1(I) collagen gene. Mutant mice homozygous for the null mutation produce no type I collagen and die at mid-gestation, whereas heterozygotes survive to adulthood. Dermal fibroblasts from heterozygous mice produce approximately 50% less type I collagen than normal littermates, and the partial deficiency in collagen production results in a phenotype similar to osteogenesis imperfecta type I (an inherited form of skeletal fragility). In this study, we have identified an adaptation of Mov13 skeletal tissue that significantly improves the bending strength of long bone. The adaptive response occurred over a 2-mo period, during which time a small number of newly proliferated osteogenic cells produced a significant amount of matrix components and thus generated new bone along periosteal surfaces. New bone deposition resulted in a measurable increase in cross-sectional geometry which, in turn, led to a dramatic increase in long bone bending strength.