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首页> 外文期刊>Oral diseases >Expression and distribution of SIBLING proteins in the predentin/dentin and mandible of hyp mice.
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Expression and distribution of SIBLING proteins in the predentin/dentin and mandible of hyp mice.

机译：SIBLING蛋白在hydent小鼠的predentin / dentin和下颌骨中的表达和分布。

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OBJECTIVES: Human X-linked hypophosphatemia (XLH) and its murine homologue, Hyp are caused by inactivating mutations in PHEX gene. The protein encoded by PHEX gene is an endopeptidase whose physiological substrate(s) has not been identified. Dentin matrix protein 1 (DMP1) and dentin sialophosphoprotein (DSPP), two members of the Small Integrin-Binding LIgand, N-linked Glycoprotein (SIBLING) family are proteolytically processed. It has been speculated that PHEX endopeptidase may be responsible for the proteolytic cleavage of DMP1 and DSPP. To test this hypothesis and to analyse the distribution of SIBLING proteins in the predentin/dentin complex and mandible of Hyp mice, we compared the expression of four SIBLING proteins, DMP1, DSPP, bone sialoprotein (BSP) and osteopontin (OPN) between Hyp and wild-type mice. METHODS: These SIBLING proteins were analysed by protein chemistry and immunohistochemistry. RESULTS: (1) Dentin matrix protein 1 and DSPP fragments are present in the extracts of Hyp predentin/dentin and bone; (2) the level of DMP1 proteoglycan form, BSP and OPN is elevated in the Hyp bone. CONCLUSIONS: The PHEX protein is not the enzyme responsible for the proteolytic processing of DMP1 and DSPP. The altered distribution of SIBLING proteins may be involved in the pathogenesis of bone and dentin defects in Hyp and XLH.
机译：目的：人类X连锁低磷血症（XLH）及其鼠类同系物Hyp是由失活的PHEX基因突变引起的。 PHEX基因编码的蛋白质是一种内肽酶，其生理底物尚未被鉴定。牙本质基质蛋白1（DMP1）和牙本质唾液磷蛋白（DSPP）是小整合素结合配体的两个成员，N联糖蛋白（SIBLING）家族经过蛋白水解处理。据推测，PHEX内肽酶可能负责DMP1和DSPP的蛋白水解切割。为了验证这一假设并分析SIBLING蛋白在Hyp小鼠前体素/牙本质复合物中和下颌骨中的分布，我们比较了Hyp和Hep之间四种SIBLING蛋白DMP1，DSPP，骨唾液蛋白（BSP）和骨桥蛋白（OPN）的表达。野生型小鼠。方法：通过蛋白质化学和免疫组织化学分析这些SIBLING蛋白。结果：（1）Hyp predentin / dentin和骨骼的提取物中存在牙本质基质蛋白1和DSPP片段; （2）Hyp骨中DMP1蛋白聚糖形式，BSP和OPN的水平升高。结论：PHEX蛋白不是负责DMP1和DSPP蛋白水解过程的酶。 SIBLING蛋白分布的改变可能与Hyp和XLH中骨骼和牙本质缺损的发病机理有关。

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