Mineralized tissue protein profiles in the Australian form of bovine osteogenesis imperfecta.
Noncollagenous proteins and proteoglycan from the bone and dentin of affected and normal half-siblings neonatal calves with the Australian variant of bovine osteogenesis imperfecta (BOI-A) were quantitated. In contrast to a clinically similar syndrome of BOI in the progeny of a second progenitor bull identified in Texas (BOI-T), osteonectin and bone sialoprotein levels were normal in the affected BOI-A bone, and dentin phosphophoryn levels were normal in affected BOI-A teeth. However, bone proteoglycan in the affected BOI-A calves was depleted to one-third of the level found in normal siblings, a finding qualitatively similar to the depletion of bone proteoglycan in the BOI-T syndrome. These results suggest that quantitation of noncollagenous proteins and proteoglycans may be a useful technique for differentiating clinically similar syndromes of OI in man.
Fisher
L W
LW
Denholm
L J
LJ
Conn
K M
KM
Termine
J D
JD
D000818:Animals; D001315:Australia; D001842:Bone and Bones / Q000032:analysis*; D002352:Carrier Proteins / Q000032:analysis; D002417:Cattle; D002418:Cattle Diseases / Q000378:metabolism*; D002848:Chromatography, DEAE-Cellulose; D003804:Dentin / Q000032:analysis*; D004195:Disease Models, Animal; D004591:Electrophoresis, Polyacrylamide Gel; D004797:Enzyme-Linked Immunosorbent Assay; D008903:Minerals / Q000032:analysis; D010013:Osteogenesis Imperfecta / Q000378:metabolism / Q000662:veterinary*; D015676:Osteonectin; D010750:Phosphoproteins / Q000032:analysis; D011506:Proteins / Q000032:analysis*; D011509:Proteoglycans / Q000032:analysis
D002352:Carrier Proteins; D008903:Minerals; D015676:Osteonectin; D010750:Phosphoproteins; D011506:Proteins; D011509:Proteoglycans; C017630:phosphophoryn