Summary
Samples of human osteogenesis imperfecta (OI) bone were analyzed for osteonectin content by SDS gel electrophoresis and immunodetection on Western blots. The OI bone osteonectin content varied from normal to severely depressed. Previously, we showed that two clinically identical but genetically unrelated bovine methods of OI were differentiated biochemically by their bone osteonectin content: one OI mode had normal bone osteonectin while the other was severely depressed in this parameter. The data in this pilot study suggest that further investigation of bone osteonectin content may prove useful in the clinical assessment of human OI cases.
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References
McKusick, VA (1972) Heritable disorders of connective tissue, 4th ed. C.V. Mosby, St. Louis, pp 390–454
Sillence DO, Senn AS, Danks DM (1979) Genetic heterogeneity in osteogenesis imperfecta. J Med Genet 16:101–106
Sillence DO (1981) Osteogenesis imperfecta; an expanding panorma of variants. Clin Orthop 159:11–25
Byers PH, Shapiro JR, Rowe DW, David KE, Holbrook KA (1983) Abnormal alpha 2-chain in type I collagen from a patient with a form of osteogenesis imperfecta. J Clin Invest 71:689–697
Williams CJ, Prockop DJ (1983). Synthesis and processing of a type I procollagen containing shortened pro-alpha 1(I) chains by fibroblasts from a patient with osteogenesis imperfecta. J Biol Chem 258:5915–5921
deWet WJ, Pihlajaniemi T, Myers J, Kelly T, Prockop DJ (1983) Synthesis of a shortened pro-alpha 2(I) chain in a proband with osteogenesis imperfacta. J Biol Chem 258:7721–7728
Pope FM, Cheah KSE, Nicholls AC, Price AB, Grosveld FG (1984) Lethal osteogenesis imperfacta congenita and a 300 base pair gene deletion for a alpha 1(I)-like collagen. Br Med J 288:431–434
Steinmann B, Rao VH, Vogel A, Bruckner P, Gitzelmann R, Byers PH (1984) Cysteine in the triple-helical domain of one allelic product of the α1(I) gene of type I collagen produces a lethal form of osteogenesis imperfacta. J Biol Chem 259:11129–11138
Chu M-L, Williams CJ, Pepe G, Hirsch JL, Prockop DJ, Ramirez F (1983) Internal delection in a collagen gene in a perinatal lethal form of osteogenesis imperfecta. Nature 304:78–80
Dickson LA, Pihlajaniemi T, Deak SB, Pope FM, Nicholls AC, Prockop DJ, Meyers JC (1984) Nuclease S1 mapping of a homozygous mutation in the carboxy-propeptide coding region of the pro α2(I) collagen gene in a patient with osteogenesis imperfecta. Proc Natl Acad Sci USA 81:4524–4528
Sykes B, Francis MJO, Smith R (1977) Altered relation of two collagen types in osteogenesis imperfecta. N Engl J Med 296:1200–1203
deVries WN, deWet WJ (1986) The molecular defect in an autosomal dominant form of osteogenesis imperfecta. J Biol Chem 261:9056–9064
Steinmann B, Nicholls A, Pope FM (1986) Clinical variability of osteogenesis imperfecta reflecting molecular heterogeneity: cysteine substitutions in the α1(I) collagen chain producing lethal and mild forms. J Biol Chem 261:8958–8964
Bonadio J, Byers PH (1985) Subtle structural alterations in the chains of type I procollagen produce osteogenesis imperfecta type II. Nature 316:363–366
Minor RR, Sippola-Thiele M, McKeon J, Berger J, Prockop DJ (1986) Defects in the processing of procollagen to collagen are demonstrable in cultured fibroblasts from patients with the Ehlers-Danlos and osteogenesis imperfacta syndromes. J Biol Chem 261:10006–10014
Denholm LJ, Cole WG (1983) Heritable bone fragility, joint laxity and dysplastic dentin in Friesian calves: a bovine syndrome of osteogenesis imperfecta. Aust Vet J 60:9–17
Fisher LW, Denholm LJ, Conn KM, Termine JD (1986) Mineralized tissue protein profiles in the Australian form of bovine osteogenesis imperfecta. Calcif Tissue Int 38:16–20
Termine JD, Gehron Robey P, Fisher LW, Shimokawa H, Drum MA, Conn KM, Hawkins GR, Cruz JB, Thompson KG (1984) Osteonectin, bone proteoglycan, and phosphophoryn defects in a form of bovine osteogenesis imperfecta. Proc Natl Acad Sci USA 81:2213–2217
Minor RR, Pihlajaniemi T, Prockop DJ, Denholm LJ, Wootton JAM (1984) Post-translational over-modification of type I, type III and type V collagens in bovine and human osteogenesis imperfecta. J Cell Biol 99:405a
Termine JD, Belcourt AB, Conn KM, Kleinman HK (1981) Mineral and collagen-binding proteins of fetal calf bone. J Biol Chem 256:10403–10408
Termine JD, Kleinman HK, Whitson SW, Conn KM, McGravey ML, Martin GR (1981) Osteonectin, a bone-specific protein linking mineral to collagen. Cell 26:99–105
Romberg RW, Werness PG, Lollar P, Riggs BL, Mann KG (1985) Isolation and characterization of native adult osteonectin. J Biol Chem 260:2728–2736
Gehron Robey P, Fisher LW, Stubbs JT, Termine JD (in press) Biosynthesis of osteonectin and a small proteoglycan (PG-II) by connective tissue cells in vitro. UCLA Symposium on Molecular and Cellular Biology, New Series
Gehron Robey P, Termine JD (1985) Human bone cells in vitro. Calcif Tissue Int 37:453–460
Termine JD, Belcourt AB, Christner DJ, Conn KM, Nylen MU (1980) Properties of dissociatively extracted fetal tooth matrix proteins. J Biol Chem 255:9760–9768
Termine JD (1983) Osteonectin and other newly described proteins of developing bone. In: Peck, William A (ed) Bone and mineral research. Excerpta Medica, Amsterdam, pp 144–156
Laemmli UK (1970) Cleavage of structural proteins during the assembly of the head of bacteriophate T4. Nature 227:680–685
Fisher LW, Termine JD, Dejter SW, Whitson SW, Yanagishita M, Kumura JH, Hascall VC, Kleinman HK, Hassell JR, Nilsson BO (1983) Proteoglycans of developing bone. J Biol Chem 258:6588–6594
Towbin H, Stehelin T, Gordon J (1979) Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications. Proc Natl Acad Sci USA 76:4350–4354
Fisher LW, Whitson SW, Avioli LV, Termine JD (1983) Matrix sialoprotein of developing bone. J Biol Chem 258:12723–12727
Conn KM, Termine JD (1985) Matrix protein profiles in calf bone development bone. Bone 6:33–36
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Fisher, L.W., Drum, M.A., Robey, P.G. et al. Osteonectin content in human osteogenesis imperfecta bone shows a range similar to that of two bovine models of OI. Calcif Tissue Int 40, 260–264 (1987). https://doi.org/10.1007/BF02555258
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DOI: https://doi.org/10.1007/BF02555258