Comparison of bone tissue properties in mouse models with collagenous and non-collagenous genetic mutations using FTIRI.

Comparison of bone tissue properties in mouse models with collagenous and non-collagenous genetic mutations using FTIRI.
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DOI:
10.1016/j.bone.2012.08.110
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发表时间:
2012-11
期刊:
影响因子:
4.1
通讯作者:
Boskey, Adele
Boskey, Adele
中科院分区:
医学2区
文献类型:
--
作者:
Coleman, Rhima M.;Aguilera, Laura;Quinones, Layla;Lukashoya, Lyudamila;Poirier, Christophe;Boskey, Adele

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了解各种形式的成骨细胞(OI)的骨组织的材料特性如何不同,将使我们能够为受影响的患者量身定制治疗方案。为此,我们的特点是骨结构和材料特性的两个小鼠模型的OI,成骨细胞的小鼠(oim/oim)和fragilitas ossium(fro/fro),其中骨脆性是由于遗传缺陷的I型胶原和成骨细胞基质矿化的缺陷,分别。使用傅里叶变换红外光谱成像(FTIRI)、显微计算机断层扫描(micro-CT)、组织学和生化分析检查3至6个月大动物的骨骼。与野生型动物相比,oim/oim骨组织的属性随时间推移相对恒定。oim/oim皮质和骨小梁中的矿物质密度高于野生型,而骨具有更薄的皮质和更薄且更宽间隔的更少的骨小梁。来回动物在3个月时表现出骨质减少属性。然而,到6个月时,它们的光谱和几何性质与野生型动物相似。尽管在fro/fro小鼠中缺乏特定的胶原蛋白缺陷,但fro/fro和oim/oim基因型在两个时间点都表现出异常的胶原蛋白交联,如通过FTIRI所确定的。这些结果表明,异常的细胞外基质组装在这两种模型中的骨脆性中起作用。
Understanding how the material properties of bone tissue from the various forms of osteogenesis imperfecta (OI) differ will allow us to tailor treatment regimens for affected patients. To this end, we characterized the bone structure and material properties of two mouse models of OI, the osteogenesis imperfecta mouse (oim/oim) and fragilitas ossium (fro/fro), in which bone fragility is due to a genetic defect in collagen type I and a defect in osteoblast matrix mineralization, respectively. Bones from 3 to 6 month old animals were examined using Fourier transform infrared spectroscopic imaging (FTIRI), microcomputed tomography (micro-CT), histology, and biochemical analysis. The attributes of oim/oim bone tissue were relatively constant over time when compared to wild type animals. The mineral density in oim/oim cortices and trabecular bone was higher than wild type while the bones had thinner cortices and fewer trabeculae that were thinner and more widely spaced. The fro/fro animals exhibited osteopenic attributes at 3 months. However, by 6 months, their spectroscopic and geometric properties were similar to wild type animals. Despite the lack of a specific collagen defect in fro/fro mice, both fro/fro and oim/oim genotypes exhibited abnormal collagen crosslinking as determined by FTIRI at both time points. These results demonstrate that abnormal extracellular matrix assembly plays a role in the bone fragility in both of these models.
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