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Morphological assessment of bone mineralization in tibial metaphyses of ascorbic acid-deficient ODS rats

机译:抗坏血酸缺乏型ODS大鼠胫骨干phy端骨矿化的形态学评估

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Osteogenic disorder shionogi (ODS) rats carry a hereditary defect in ascorbic acid synthesis, mimicking human scurvy when fed with an ascorbic acid-deficient (aa-def) diet. As aa-def ODS rats were shown to feature disordered bone formation, we have examined the bone mineralization in this rat model. A fibrous tissue layer surrounding the trabeculae of tibial metaphyses was found in aa-def ODS rats, and this layer showed intense alkaline phosphatase activity and proliferating cell nuclear antigen-immunopositivity. Many osteoblasts detached from the bone surfaces and were characterized by round-shaped rough endoplasmic reticulum (rER), suggesting accumulation of malformed collagen inside the rER. Accordingly, fine, fragile fibrillar collagenous structures without evident striation were found in aa-def bones, which may result from misassembling of the triple helices of collagenous α-chains. Despite a marked reduction in bone formation, ascorbic acid deprivation seemed to have no effect on mineralization: while reduced in number, normal matrix vesicles and mineralized nodules could be seen in aa-def bones. Fine needle-like mineral crystals extended from these mineralized nodules, and were apparently bound to collagenous fibrillar structures. In summary, collagen mineralization seems unaffected by ascorbic acid deficiency in spite of the fine, fragile collagenous fibrils identified in the bones of our animal model.
机译:成骨性疾病shionogi(ODS)大鼠在抗坏血酸合成方面存在遗传缺陷,在喂食抗坏血酸缺乏(aa-def)饮食时模仿人类坏血病。由于aa-def ODS大鼠显示出异常的骨形成特征,因此我们检查了该大鼠模型中的骨矿化。在aa-def ODS大鼠中发现了围绕胫骨干meta小梁的纤维组织层,该层显示出强烈的碱性磷酸酶活性和增殖的细胞核抗原免疫阳性。许多成骨细胞从骨表面脱落,并具有圆形粗糙的内质网(rER)的特征,表明rER内积累了畸形胶原。因此,在aa-def骨骼中发现了细小,脆弱的原纤维胶原结构,没有明显的条纹,这可能是由于胶原α链的三重螺旋错配造成的。尽管骨骼形成明显减少,但抗坏血酸的缺乏似乎对矿化没有影响:虽然数量减少,但在aa-def骨骼中可以看到正常的基质囊泡和矿化的结节。细针状矿物晶体从这些矿化的结节中延伸出来,并且显然与胶原性纤维状结构结合在一起。总之,尽管在我们动物模型的骨骼中发现了细小,脆弱的胶原纤维,但胶原矿化似乎不受抗坏血酸缺乏的影响。

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