title: Osteogenesis imperfecta: changes in noncollagenous proteins in bone.
abstract: 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.
authors.affiliation: National Institutes of Health, National Institute of Dental Research, Bone Research Branch, Bethesda, MD 20892.
authors.forename: U
authors.initials: U
authors.lastname: Vetter
authors.forename: L W
authors.initials: LW
authors.lastname: Fisher
authors.forename: K P
authors.initials: KP
authors.lastname: Mintz
authors.forename: J B
authors.initials: JB
authors.lastname: Kopp
authors.forename: N
authors.initials: N
authors.lastname: Tuross
authors.forename: J D
authors.initials: JD
authors.lastname: Termine
authors.forename: P G
authors.initials: PG
authors.lastname: Robey
mesh_terms: D000293:Adolescent; D000596:Amino Acids / Q000032:analysis; D001798:Blood Proteins / Q000378:metabolism; D015519:Bone Density; D001842:Bone and Bones / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D058575:Decorin; D016326:Extracellular Matrix Proteins; D006801:Humans; D007223:Infant; D015675:Osteocalcin / Q000378:metabolism; D010013:Osteogenesis Imperfecta / Q000378:metabolism*; D015676:Osteonectin / Q000378:metabolism; D011506:Proteins / Q000378:metabolism*; D011509:Proteoglycans / Q000378:metabolism; D012795:Sialoglycoproteins / Q000378:metabolism; D060749:alpha-2-HS-Glycoprotein
chemical_list: C556354:AHSG protein, human; D000596:Amino Acids; D001798:Blood Proteins; C546878:DCN protein, human; D058575:Decorin; D016326:Extracellular Matrix Proteins; D015676:Osteonectin; D011506:Proteins; D011509:Proteoglycans; D012795:Sialoglycoproteins; D060749:alpha-2-HS-Glycoprotein; D015675:Osteocalcin