Vetter U, Fisher L W, Mintz K P, Kopp J B, Tuross N, Termine J D, Robey P G
National Institutes of Health, National Institute of Dental Research, Bone Research Branch, Bethesda, MD 20892.
J Bone Miner Res. 1991 May;6(5):501-5. doi: 10.1002/jbmr.5650060512.
The noncollagenous proteins osteonectin, bone sialoprotein, osteocalcin, the small proteoglycan decorin (PG II), and alpha 2-HS glycoprotein (which is synthesized in the liver but highly concentrated in bone) were measured in extracts of cortical bone from 3 type I, 2 type II, 8 type III and 13 type IV patients with osteogenesis imperfecta (OI) and from 7 control subjects. Osteonectin was found to be reduced in the bone of all OI patients. The bone from severely affected type III OI patients contained the lowest levels of osteonectin. In contrast, bone sialoprotein was found to be elevated in the bones of OI patients. The highest levels were found in individuals classified as type IV patients. Osteocalcin and alpha 2-HS glycoprotein concentrations were increased in all OI patients. Decorin levels were not significantly altered in OI bones compared to controls. These changes in the concentrations of the noncollagenous proteins may contribute to the fragility of the OI bone by interfering with complete mineralization and/or normal tissue architecture.
对3例Ⅰ型、2例Ⅱ型、8例Ⅲ型和13例Ⅳ型成骨不全（OI）患者以及7名对照者的皮质骨提取物中的非胶原蛋白骨连接蛋白、骨涎蛋白、骨钙素、小分子蛋白聚糖核心蛋白聚糖（PG II）和α2 - HS糖蛋白（在肝脏中合成但在骨中高度浓缩）进行了检测。发现所有OI患者骨中的骨连接蛋白均减少。严重受累的Ⅲ型OI患者的骨中骨连接蛋白水平最低。相比之下，发现OI患者骨中的骨涎蛋白升高。在分类为Ⅳ型患者的个体中发现最高水平。所有OI患者的骨钙素和α2 - HS糖蛋白浓度均升高。与对照组相比，OI骨中的核心蛋白聚糖水平没有显著变化。这些非胶原蛋白浓度的变化可能通过干扰完全矿化和/或正常组织结构而导致OI骨的脆性增加。
