Mineral Changes in a Mouse Model of Osteogenesis Imperfecta Detected by Fourier Transform Infrared Microscopy
- 1 January 1996
- journal article
- research article
- Published by Taylor & Francis in Connective Tissue Research
- Vol. 35 (1-4) , 259-265
- https://doi.org/10.3109/03008209609029199
Abstract
Osteogenesis imperfecta (OI) is a heritable disease characterized by skeletal deformities and brittle bones. In the current study, the nature of the mineral in long bones of a mouse model of OI (pim/oitn, a mutant which produces an α1(I) collagen homotrimer) was examined by Fourier transform infrared microscopy. The minerahmatrix ratio of oim/oim cortical bone was greater than that of the heterozygous oim/+ and of the normal +/+ bones, probably as a result of reduced collagen content. The molecular environments of the apatitic phosphates differed among the oim/oim and the oim/+ and the +/+ bones. This was attributable to several factors, including dissimilar mineral-matrix interactions and differences in the chemical composition of the mineral. It was concluded from these data that the defective collagen matrix leads to abnormal mineral formation at the molecular level and thus results in tissues with reduced mechanical properties.Keywords
This publication has 20 references indexed in Scilit:
- An electron probe X-ray microanalytical study of bone mineral in osteogenesis imperfectaCalcified Tissue International, 1995
- Crystal and Molecular Structure of a Collagen-Like Peptide at 1.9 Å ResolutionScience, 1994
- Ultrastructural studies of bones from patients with osteogenesis imperfectaMatrix Biology, 1994
- Delayed Triple Helix Formation of Mutant Collagen from Patient with Osteogenesis ImperfectaJournal of Molecular Biology, 1994
- Defective pro alpha 2(I) collagen synthesis in a recessive mutation in mice: a model of human osteogenesis imperfecta.Proceedings of the National Academy of Sciences, 1993
- Transgenic mice expressing a partially deleted gene for type I procollagen (COL1A1). A breeding line with a phenotype of spontaneous fractures and decreased bone collagen and mineral.Journal of Clinical Investigation, 1993
- Collagen crosslinks and mineral crystallinity in bone of patients with osteogenesis imperfectaJournal of Bone and Mineral Research, 1993
- OUR UNDERSTANDING OF INHERITED SKELETAL FRAGILITY AND WHAT THIS HAS TAUGHT US ABOUT BONE STRUCTURE AND FUNCTIONPublished by Elsevier ,1993
- Research Perspectives in Heritable Disorders of Connective TissueMatrix, 1992
- Changes in apatite crystal size in bones of patients with osteogenesis imperfectaCalcified Tissue International, 1991