Abstract
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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
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)
Epstein, E. H. Jr.: (α1(III))3 Human skin collagen. J. Biol. Chem. 249, 3225–3231 (1974)
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)
McKusick, V. A.: In: “Heritable Disorders of Connective Tissue”, pp. 390–441. Saint Louis, USA: The C. V. Mosby Company 1972
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)
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)
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)
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)
Piez, K. A.: Molecular weight determination of random coil polypeptides from collagen by molecular sieve chromatography. Anal. Biochem. 26, 305–312 (1968)
Pontz, B. F., Müller, P. K., Meigel, W. N.: A study on the conversion of procollagen. J. Biol. Chem. 248, 7558–7564 (1973)
Teitelbaum, S. L., Kraft, W. J., Avioli, L. V.: Bone collagen aggregation abnormalities in osteogenesis imperfecta. Calcif. Tissue Res. 17, 75–79 (1974)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Müller, P.K., Raisch, K., Matzen, K. et al. Presence of type III collagen in bone from a patient with osteogenesis imperfecta. Eur J Pediatr 125, 29–37 (1977). https://doi.org/10.1007/BF00470603
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00470603