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在牙本质,两个编码I型胶原蛋白基因的突变会导致成骨发育不全。除了胶原蛋白,在牙本质还有一定数量的非胶原蛋白。在牙本质非胶原蛋白编码的基因中,只有牙本质唾液酸焦磷酸蛋白(dentin sialophosphoprotein,DSPP)的突变引起遗传性牙齿畸形。DSPP的突变会引起牙本质发育不全(dentinogenesis imperfecta,DGI)Ⅰ型、Ⅱ型和牙本质发育异常(dentin dysplasia,DD)Ⅱ型。DSPP由成牙质细胞表达并分泌。分泌之后,DSPP由多个细胞外蛋白酶酶切成小片。DSPP被蛋白酶酶切为三个主要部分:牙本质涎蛋白(dentin sialoprotein,DSP),牙本质糖蛋白(dentin glycoprotein,DGP)和牙本质磷蛋白(dentin phosphoprotein,DPP)。本文就这三种蛋白的最新进展进行回顾。
In dentin, two mutations that encode a type I collagen gene result in azooblastogenesis. In addition to collagen, there is a certain amount of non-collagenous protein in the dentine. Of dentin non-collagen encoding genes, only mutations in dentin sialophosphoprotein (DSPP) cause genetic tooth deformities. DSPP mutations cause dentino dysplasia (DGI) type I, type II and dentin dysplasia (DD) type II. DSPP is expressed and secreted by odontoblasts. After secretion, DSPP is digested by multiple extracellular proteases into small pieces. DSPP is proteolytically digested into three major components: dentin sialoprotein (DSP), dentin glycoprotein (DGP), and dentin phosphoprotein (DPP). This article reviews the recent advances in these three proteins.