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低磷血症小鼠骨骼中的磷脂变化

Phospholipid changes in the bones of the hypophosphatemic mouse.

作者信息

Boskey A L, Gilder H, Neufeld E, Ecarot B, Glorieux F H

机构信息

Hospital for Special Surgery, New York, NY.

出版信息

Bone. 1991;12(5):345-51. doi: 10.1016/8756-3282(91)90021-a.

DOI:10.1016/8756-3282(91)90021-a
PMID:1782102
Abstract

The mineral and lipid composition of the bones of 35 day old hypophosphatemic (Hyp/Y) and control (+/Y) mice were compared in order to test the hypothesis that phosphate status has an effect on the complexed acidic phospholipid content of developing bones. The Hyp/Y bones were found to be rachitic and osteomalacic, having significantly reduced mineral content. That mineral was shown by X-ray diffraction to consist of larger/more perfect crystals than that in +/Y animals, indicating either a preference for crystal growth rather than new mineral deposition, or an increased mineral turnover. The increased crystal perfection was confirmed by chemical analyses which showed an increased calcium to phosphorus ratio in the Hyp/Y bones. The bones of Hyp/Y animals had significantly reduced complexed acidic phospholipid contents relative to those of control animals. Since these complexes are believed to play a role in vitro and in vivo mineral deposition, it is suggested that the deficiency of these complexes contributes to the mineralization defect. The magnitude of the complexed acidic phospholipid deficiency in the Hyp/Y animals indicates the importance of phosphate for the formation of these lipids. Although the proportion of phosphatidylserine and phosphatidylinositol and lysophospholipids tended to be reduced in the Hyp/Y bones, the absence of other statistically significant phospholipid abnormalities in the bones and brains of these animals suggests that the lipid defect is not systemic, but is associated with a decrease in phosphate at the site of bone formation.

摘要

为了验证磷酸盐状态对发育中骨骼的复合酸性磷脂含量有影响这一假设,对35日龄的低磷血症(Hyp/Y)小鼠和对照(+/Y)小鼠的骨骼矿物质和脂质成分进行了比较。发现Hyp/Y小鼠的骨骼患有佝偻病和骨软化症,矿物质含量显著降低。X射线衍射显示,该矿物质由比+/Y动物更大/更完美的晶体组成,这表明要么是晶体生长优于新矿物质沉积,要么是矿物质周转率增加。化学分析证实了晶体完美度的增加,分析显示Hyp/Y小鼠骨骼中的钙磷比增加。与对照动物相比,Hyp/Y动物骨骼中的复合酸性磷脂含量显著降低。由于这些复合物被认为在体外和体内矿物质沉积中起作用,因此表明这些复合物的缺乏导致了矿化缺陷。Hyp/Y动物中复合酸性磷脂缺乏的程度表明磷酸盐对这些脂质形成的重要性。尽管Hyp/Y小鼠骨骼中磷脂酰丝氨酸、磷脂酰肌醇和溶血磷脂的比例趋于降低,但这些动物骨骼和大脑中没有其他具有统计学意义的磷脂异常,这表明脂质缺陷不是全身性的,而是与骨形成部位的磷酸盐减少有关。

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Expression and distribution of SIBLING proteins in the predentin/dentin and mandible of hyp mice.SIBLING 蛋白在 hyp 小鼠牙本质/牙髓和下颌骨中的表达和分布。
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The PHEX transgene corrects mineralization defects in 9-month-old hypophosphatemic mice.PHEX转基因可纠正9月龄低磷血症小鼠的矿化缺陷。
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Abnormal mineral-matrix interactions are a significant contributor to fragility in oim/oim bone.异常的矿物质-基质相互作用是oim/oim小鼠骨骼脆性的一个重要因素。
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