Musculoskeletal Integrity Program, Hospital for Special Surgery, New York, NY 10021, USA.
Biomed Res Int. 2013;2013:295812. doi: 10.1155/2013/295812. Epub 2013 May 29.
The Brtl/+ mouse is a knock-in model for osteogenesis imperfecta type IV in which a Gly349Cys substitution was introduced into one COL1A1 allele. To gain insight into the changes in dentin structure and mineral composition in these transgenic mice, the objective of this study was to use microcomputed tomography (micro-CT), scanning electron microscopy (SEM), and Fourier transform infrared imaging (FTIRI) to analyze these structures at 2 and 6 months of age. Results, consistent with the dental phenotype in humans with type IV OI, showed decreased molar volume and reduced mineralized tissue volume in the teeth without changes in enamel properties. Increased acid phosphate content was noted at 2 and 6 months by FTIRI, and a trend towards altered collagen structure was noted at 2 but not 6 months in the Brtl/+ teeth. The increase in acid phosphate content suggests a delay in the mineralization process, most likely associated with the defect in the collagen structure. It appears that in the Brtl/+ teeth slow maturation of the mineralized structures allows correction of altered mineral content and acid phosphate distribution.
Brtl/+ 小鼠是一种 IV 型成骨不全症的基因敲入模型,其中一个 COL1A1 等位基因引入了 Gly349Cys 取代。为了深入了解这些转基因小鼠牙本质结构和矿物质组成的变化,本研究旨在使用微计算机断层扫描(micro-CT)、扫描电子显微镜(SEM)和傅里叶变换红外成像(FTIRI)分析 2 个月和 6 个月大时的这些结构。结果与 IV 型 OI 患者的牙表型一致,显示磨牙体积减小,矿化组织体积减少,而牙釉质特性没有变化。FTIRI 显示 2 个月和 6 个月时酸性磷酸盐含量增加,Brtl/+ 牙齿在 2 个月时胶原结构发生改变的趋势,但 6 个月时没有改变。酸性磷酸盐含量的增加表明矿化过程延迟,很可能与胶原结构缺陷有关。似乎在 Brtl/+ 牙齿中,矿化结构的缓慢成熟允许纠正改变的矿物质含量和酸性磷酸盐分布。