Traub W, Arad T, Vetter U, Weiner S
Department of Structural Biology, Weizmann Institute of Science, Rehovot, Israel.
Matrix Biol. 1994 Aug;14(4):337-45. doi: 10.1016/0945-053x(94)90200-3.
Bone samples from patients suffering from osteogenesis imperfecta (OI) types I, II, III and IV, as well as normal controls, were studied by scanning (SEM) and transmission electron microscopy (TEM). SEM views of normal bone at low magnification show coherent structure, with regular striations due to a lamellar plywood-like arrangement of the mineralized collagen fibrils. Compact lamellar bone was also found in various OI specimens, but in limited disconnected regions separated by open spaces. Furthermore, some OI, but not normal, bones have regions of loose unconnected fibers and others of apparently abnormally dense mineral deposition. High resolution TEM studies of OI bone fragments have served to elucidate the structures of these different textures. There appears to be a substantial, though reduced, proportion of normal lamellar bone even in quite severe OI. However, the regions of loose fibers are largely unmineralized and probably contain abnormal collagen. Other regions are overmineralized, with generally small unorganized apatite crystals deposited onto fibril surfaces or in separate clusters. These structural abnormalities, together with the paucity of normal bone, may explain the fragility of OI bones.
对患有I型、II型、III型和IV型成骨不全症（OI）的患者以及正常对照的骨样本进行了扫描电子显微镜（SEM）和透射电子显微镜（TEM）研究。低倍放大下正常骨的SEM图像显示结构连贯，由于矿化胶原纤维呈层状胶合板样排列而有规则的条纹。在各种OI标本中也发现了致密的层状骨，但存在于由开放空间分隔的有限不连续区域。此外，一些OI骨（而非正常骨）有松散的未连接纤维区域，以及其他明显矿化异常致密的区域。对OI骨碎片的高分辨率TEM研究有助于阐明这些不同纹理的结构。即使在相当严重的OI中，似乎也有相当比例（尽管有所减少） 的正常层状骨。然而，松散纤维区域大多未矿化，可能含有异常胶原。其他区域矿化过度，通常有小的无组织磷灰石晶体沉积在纤维表面或单独的簇中。这些结构异常，连同正常骨的缺乏，可能解释了OI骨的脆弱性。
