The Role of DSPP in Dentine Formation and Hereditary Dentine Defects.

Jie Jia, Zhuan Bian, Yaling Song
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Abstract

The dentine sialophosphoprotein (DSPP) gene is the only identified causative gene for dentinogenesis imperfecta type 2 (DGI-II), dentinogenesis imperfecta type 3 (DGI-III) and dentine dysplasia type 2 (DD-II). These three disorders may have similar molecular mechanisms involved in bridging the DSPP mutations and the resulting abnormal dentine mineralisation. The DSPP encoding proteins DSP (dentine sialoprotein) and DPP (dentine phosphoprotein) are positive regulators of dentine formation and perform a function during dentinogenesis. The present review focused on the recent findings and viewpoints regarding the relationship between DSPP and dentinogenesis as well as mineralisation from multiple perspectives, involving studies relating to spatial structure and tissue localisation of DSPP, DSP and DPP, the biochemical characteristics and biological function of these molecules, and the causative role of the proteins in phenotypes of the knockout mouse model and in hereditary dentine defects.

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DSPP 在牙本质形成和遗传性牙本质缺陷中的作用
牙本质苷磷蛋白(DSPP)基因是牙本质发育不全 2 型(DGI-II)、牙本质发育不全 3 型(DGI-III)和牙本质发育不良 2 型(DD-II)的唯一已确定的致病基因。这三种疾病可能具有类似的分子机制,参与连接 DSPP 突变和由此导致的牙本质矿化异常。DSPP 编码蛋白 DSP(牙本质硫蛋白)和 DPP(牙本质磷蛋白)是牙本质形成的正调控因子,在牙本质形成过程中发挥功能。本综述侧重于从多个角度探讨 DSPP 与牙本质生成和矿化之间关系的最新发现和观点,涉及 DSPP、DSP 和 DPP 的空间结构和组织定位相关研究、这些分子的生化特征和生物功能,以及这些蛋白在基因敲除小鼠模型表型和遗传性牙本质缺陷中的致病作用。
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