References
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MGI Accession ID: MGI:3611515
J Number: J:104207
Other Accession IDs:
Title: A murine skeletal adaptation that significantly increases cortical bone mechanical properties. Implications for human skeletal fragility.
Authors: Bonadio J; Jepsen KJ; Mansoura MK; Jaenisch R; Kuhn JL; Goldstein SA
Journal: J Clin Invest
Volume: 92
Issue: 4
Date: 1993 Oct
Year: 1993
Pages: 1697-705
Review Status: Peer Reviewed
Abstract:
Mov13 mice carry a provirus that prevents transcription initiation of the alpha 1(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.
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