Ultrastructural studies of bones from patients with osteogenesis imperfecta.
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.
Traub
W
W
Department of Structural Biology, Weizmann Institute of Science, Rehovot, Israel.
Arad
T
T
Vetter
U
U
Weiner
S
S
D000293:Adolescent; D001842:Bone and Bones / Q000648:ultrastructure*; D002648:Child; D002675:Child, Preschool; D005269:Femur / Q000648:ultrastructure; D005315:Fetal Diseases / Q000473:pathology; D006801:Humans; D008854:Microscopy, Electron; D008855:Microscopy, Electron, Scanning; D010013:Osteogenesis Imperfecta / Q000473:pathology*; D012720:Severity of Illness Index