Camacho N P, Landis W J, Boskey A L
Research Division, Hospital for Special Surgery, New York, NY 10211, USA.
Connect Tissue Res. 1996;35(1-4):259-65. doi: 10.3109/03008209609029199.
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 (oim/oim, a mutant which produces an alpha 1(I) collagen homotrimer) was examined by Fourier transform infrared microscopy. The mineral:matrix 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.
成骨不全症（OI）是一种遗传性疾病，其特征为骨骼畸形和骨质脆弱。在当前研究中，通过傅里叶变换红外显微镜检查了成骨不全症小鼠模型（oim/oim，一种产生α1(I)胶原同三聚体的突变体）长骨中矿物质的性质。oim/oim皮质骨的矿物质与基质之比大于杂合子oim/+和正常+/+骨骼，这可能是胶原含量降低的结果。磷灰石磷酸盐的分子环境在oim/oim、oim/+和+/+骨骼之间有所不同。这归因于几个因素，包括不同的矿物质-基质相互作用以及矿物质化学成分的差异。从这些数据得出的结论是，有缺陷的胶原基质导致分子水平上矿物质形成异常，从而导致组织力学性能降低。
