Presence of type III collagen in bone from a patient with osteogenesis imperfecta
Samples of bone from a patient with osteogenesis imperfecta were found to synthesize and contain type III collagen as well as type I collagen. Normal bone contains only type I collagen except in the lining cells of the bone marrow cavities. In the patient's tissue, type III collagen was localized in nonfibrillar structures in discrete areas of the bone. These and previous studies indicate that certain types of osteogenesis imperfecta may be caused by a failure of normal bone maturation and the sites in which the type III collagen is found appear to be defects in the bone.
Key wordsOsteogenesis imperfecta Collagen types Bone In vitro study Immunofluorescence
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- Chung, E., Miller, E. J.: Collagen polymorphism: characterization of molecules with the chain composition (α1(III))3 in human tissues. Science 183, 1200–1201 (1974)Google Scholar
- Epstein, E. H. Jr.: (α1(III))3 Human skin collagen. J. Biol. Chem. 249, 3225–3231 (1974)Google Scholar
- Epstein, E. H. Jr.:, Munderloh, N. H.: Isolation and characterization of CNBr peptides of human (α1(III))3 collagen and tissue distribution of (α1(I))2α2 and (α1(III))3 collagen. J. Biol. Chem. 250, 9304–9312 (1975)Google Scholar
- McKusick, V. A.: In: “Heritable Disorders of Connective Tissue”, pp. 390–441. Saint Louis, USA: The C. V. Mosby Company 1972Google Scholar
- Müller, P. K., Lemmen, C., Gay, S., Meigel, W. N.: Disturbance in the regulation of the type of collagen synthesized in a form of osteogenesis imperfecta. Eur. J. Biochem. 59, 97–104 (1975)Google Scholar
- Nowack, H., Gay, S., Wick, G., Becker, U., Timpl, R.: Preparation and use in immunohistology of antibodies specific for type I and type III collagen and procollagen. J. Immunol. Methods 12, 117–124 (1976)Google Scholar
- Penttinen, R. P., Lichtenstein, J. R., Martin, G. R., McKusick, V. A.: Abnormal collagen metabolism in cultured cells in osteogenesis imperfecta. Proc. Nat. Acad. Sci. USA 72, 586–589 (1975)Google Scholar
- Piez, K. A., Eigner, E. A., Lewis, M. S.: The chromatographic separation and amino acid composition of the subunits of several collagens. Biochemistry 2, 58–66 (1963)Google Scholar
- Piez, K. A.: Molecular weight determination of random coil polypeptides from collagen by molecular sieve chromatography. Anal. Biochem. 26, 305–312 (1968)Google Scholar
- Pontz, B. F., Müller, P. K., Meigel, W. N.: A study on the conversion of procollagen. J. Biol. Chem. 248, 7558–7564 (1973)Google Scholar
- Teitelbaum, S. L., Kraft, W. J., Avioli, L. V.: Bone collagen aggregation abnormalities in osteogenesis imperfecta. Calcif. Tissue Res. 17, 75–79 (1974)Google Scholar