Lukinmaa P L
Department of Oral Pathology, University of Helsinki, Finland.
J Craniofac Genet Dev Biol. 1988;8(3):235-43.
Demineralized deciduous and permanent teeth from seven patients with six different types of osteogenesis imperfecta (OI) and from four unaffected controls were stained for type III collagen and for the N-terminal propeptide of type III procollagen using indirect immunofluorescence. Sillence types IA, IB and III OI were each represented by one patient. Two patients had type IVB and two had unclassifiable OI. After enzymatic treatment, the dentin matrix of one patient each with type IB OI, type IVB, and unclassifiable OI reacted with the specific antibodies against both type III collagen and the N-terminal propeptide. Positive staining was observed around the pathological canal-like structures and as delicate strands traversing the matrix. The similar patterns of immunofluorescence for both antigens in dentin in OI are suggestive of retention of the N-terminal propeptide in association with type III collagen identical to that in normal nonmineralized connective tissues. The abnormal presence of type III collagen in dentin in OI may be secondary to the aberrant structure of type I collagen. The failure of dentin matrix of all patients with OI to immunostain for type III collagen and the N-terminal propeptide may reflect heterogeneity or additional secondary changes in matrix macromolecule interactions.
对7例患有6种不同类型成骨不全症（OI）的患者以及4名未受影响的对照者的脱矿乳牙和恒牙，使用间接免疫荧光法对III型胶原蛋白和III型前胶原的N端前肽进行染色。Silence IA型、IB型和III型OI各有1例患者。2例患者为IVB型，2例为无法分类的OI。酶处理后，1例IB型OI、IVB型和无法分类的OI患者的牙本质基质与抗III型胶原蛋白和N端前肽的特异性抗体发生反应。在病理性管状结构周围以及穿过基质的细纤维中观察到阳性染色。OI患者牙本质中两种抗原的免疫荧光模式相似，提示N端前肽与III型胶原蛋白结合保留，与正常非矿化结缔组织中的情况相同。OI患者牙本质中III型胶原蛋白的异常存在可能继发于I型胶原蛋白的异常结构。所有OI患者的牙本质基质对III型胶原蛋白和N端前肽免疫染色失败，可能反映了基质大分子相互作用的异质性或其他继发性变化。
