A murine skeletal adaptation that significantly increases cortical bone mechanical properties. Implications for human skeletal fragility.

J Bonadio; K J Jepsen; M K Mansoura; R Jaenisch; J L Kuhn; S A Goldstein

doi:10.1172/JCI116756

Department of Pathology, University of Michigan, Ann Arbor 48109-0650.

Find articles by Bonadio, J. in: PubMed | Google Scholar

Department of Pathology, University of Michigan, Ann Arbor 48109-0650.

Find articles by Jepsen, K. in: PubMed | Google Scholar

Department of Pathology, University of Michigan, Ann Arbor 48109-0650.

Find articles by Mansoura, M. in: PubMed | Google Scholar

Department of Pathology, University of Michigan, Ann Arbor 48109-0650.

Find articles by Jaenisch, R. in: PubMed | Google Scholar

Department of Pathology, University of Michigan, Ann Arbor 48109-0650.

Find articles by Kuhn, J. in: PubMed | Google Scholar

Department of Pathology, University of Michigan, Ann Arbor 48109-0650.

Find articles by Goldstein, S. in: PubMed | Google Scholar

Published in Volume 92, Issue 4 on October 1, 1993
J Clin Invest. 1993;92(4):1697–1705. https://doi.org/10.1172/JCI116756.
© 1993 The American Society for Clinical Investigation

Published October 1, 1993 - Version history

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.

Images.