Fedarko N S, Robey P G, Vetter U K
Bone Research Branch, National Institute of Dental Research, National Institutes of Health, Bethesda, Maryland, USA.
J Bone Miner Res. 1995 Jul;10(7):1122-9. doi: 10.1002/jbmr.5650100718.
In previous work, we compared the steady-state levels of specific matrix components in human bone cells derived from patients with osteogenesis imperfecta (OI) to those of age-matched controls. A remarkable finding was the observation that there was a reduction not only in the total levels of collagen, but also in osteonectin and three proteoglycans (a large chondroitin sulfate proteoglycan, biglycan, and decorin). This pattern was observed in patients with and without detectable collagen defects. More recent analysis of extracellular matrix composition have yielded that, compared with age-matched controls, bone cells from OI patients produced higher steady-state levels of fibronectin and thrombospondin. The percentage of these two proteins incorporated into the cell layer pool was also higher in OI than in age-matched controls. In addition, the steady-state levels of hyaluronan and a heparan sulfate proteoglycan were analyzed in both OI and age-matched controls. Although the total (medium + cell layer) steady-state levels of hyaluronan were reduced by 1/3, the percentage of the hyaluronan in the cell layer pool of patients with OI increased between 100-250% of age-matched control. Thus the matrix elaborated by human OI bone cells is not only quantitatively different but also qualitatively distinct from that of age-matched controls. Not only have specific bone cell matrix components (collagen, osteonectin, the large chondroitin sulfate proteoglycan, biglycan, and decorin) been found to be present in reduced levels in OI bone cells, but some matrix components (thrombospondin, fibronectin, and hyaluronan) have also been found to be present in elevated levels in the matrix of OI cells.(ABSTRACT TRUNCATED AT 250 WORDS)
在之前的研究中，我们比较了成骨不全症（OI）患者来源的人骨细胞中特定基质成分的稳态水平与年龄匹配的对照组。一个显著的发现是，不仅胶原蛋白的总水平降低，骨连接蛋白和三种蛋白聚糖（一种大硫酸软骨素蛋白聚糖、双糖链蛋白聚糖和核心蛋白聚糖）的水平也降低。在有和没有可检测到的胶原蛋白缺陷的患者中均观察到这种模式。最近对细胞外基质组成的分析表明，与年龄匹配的对照组相比，OI患者的骨细胞产生的纤连蛋白和血小板反应蛋白的稳态水平更高。这两种蛋白质掺入细胞层池的百分比在OI患者中也高于年龄匹配的对照组。此外，还分析了OI患者和年龄匹配的对照组中透明质酸和一种硫酸乙酰肝素蛋白聚糖的稳态水平。尽管透明质酸的总（培养基+细胞层）稳态水平降低了1/3，但OI患者细胞层池中透明质酸的百分比增加到年龄匹配对照组的100%-250%。因此，人类OI骨细胞形成的基质不仅在数量上不同，而且在质量上也与年龄匹配的对照组不同。不仅发现特定骨细胞基质成分（胶原蛋白、骨连接蛋白、大硫酸软骨素蛋白聚糖、双糖链蛋白聚糖和核心蛋白聚糖）在OI骨细胞中的水平降低，而且还发现一些基质成分（血小板反应蛋白、纤连蛋白和透明质酸）在OI细胞的基质中的水平升高。（摘要截短至250字）
