Lukinmaa P L, Ranta H, Vaheri A
Department of Oral Pathology, University of Helsinki, Finland.
J Craniofac Genet Dev Biol. 1988;8(1):75-82.
Deciduous and permanent teeth from seven patients with five different osteogenesis imperfecta (OI) syndromes and three normal subjects were demineralized with ethanolic trimethylammonium EDTA, enzymatically pretreated and immunostained with antihuman plasma fibronectin (FN) sera. Staining for FN in the dentin matrix was positive in halo and reticular patterns in the one patient with Sillence type I B OI and in two patients of three with type IV B OI, all with dentinogenesis imperfecta (DI). The staining was negative in type I A OI without DI, in type III, in one patient with type IV B, and in an unidentified type of OI, all with DI. In normal control teeth no staining of the dentin matrix was observed. The staining differences between OI types (also with DI) may reflect genetic heterogeneity. The diverse results in type IV B OI were suggestive of interfamilial variability within the OI syndrome. The presence of FN in the dentin matrix in OI may be due to its continuous synthesis or decreased degradation during dentin development.
从7名患有5种不同成骨不全(OI)综合征的患者以及3名正常受试者身上获取乳牙和恒牙,用乙醇三甲基铵乙二胺四乙酸进行脱矿质处理,进行酶预处理,并用抗人血浆纤连蛋白(FN)血清进行免疫染色。在患有Sillence I B型OI的1名患者以及3名IV B型OI患者中的2名(均患有牙本质发育不全(DI))的牙本质基质中,FN染色呈晕圈和网状阳性。在无DI的I A型OI、III型、1名IV B型患者以及1种未明确类型的OI(均患有DI)中,染色为阴性。在正常对照牙齿中未观察到牙本质基质染色。OI各类型(也包括患有DI的类型)之间的染色差异可能反映了基因异质性。IV B型OI中的不同结果提示了OI综合征家族间的变异性。OI牙本质基质中FN的存在可能是由于其在牙本质发育过程中持续合成或降解减少。