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Summary
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The ECM contains a complex array of fibrillar and network-forming collagens, elastin fibers, a stiff gelatinous matrix of proteoglycans, and a number of glycoproteins that mediate the interaction of these molecules with one another and with the cell surface. These molecules and their interactions afford structure, stability, and elasticity to the ECM, and provide a route for communication between the intra- and extra-cellular environments in tissues. The heterogeneity of both the protein and the carbohydrate components of these molecules provides for the great diversity in the structure and function of the ECM in various tissues.
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ACTIVE LEARNING
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  1. Compare the structure of heparin, its mechanism of action, its route and frequency of administration to that of other common anticoagulants, such as aspirinView drug information and coumarin derivatives.
  2. Discuss factors that promote the turnover of ECM components, as part of normal growth and development and in diseases, such as rheumatoid arthritis.
  3. Review the consequences of genetic defects in sulfation of proteoglycans.
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Further reading
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Hulmes DJ. Building collagen molecules, fibrils, and suprafibrillar structures. J Struct Biol 2002;137(1-2):2-10. Full articleGo to this article on the publisher's site
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Aumailley M, Gayraud B. Structure and biological activity of the extracellular matrix. J Mol Med 1998;76:253-265. Full articleGo to this article on the publisher's site
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Myllyharju J, Kivirikko KI. Collagens and collagen-related diseases. Ann Med 2001;33(1):7-21.
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Brown JC, Timpl R. The collagen superfamily. Int Arch Allergy Immunol 1995;107:484-490.
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Bosman FT, Stamenkovic I. Functional structure and composition of the extracellular matrix. J Pathol 2003;200(4):423-428. Full articleGo to this article on the publisher's site
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Clark EA, Bruggse JS. Integrins and signal transduction pathways: the road taken. Science 1995;268:233-239.
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Fraser JR, Laurent TC, Laurent UB. Hyaluronan: its nature, distribution, functions and turnover. J Int Med 1997;242:27-33. Full articleGo to this article on the publisher's site
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Mewer UM, Engvall E. Domains of laminin. J Cell Biochem 1996;61:493-501.
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Prockop DJ, Kivirikko KI. Collagens: molecular biology, diseases and potentials for
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therapy. Annu Rev Biochem 1995;64:403-434.
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Rosenbloom J, Abrams WR, Mecham R. Extracellular matrix 4: the elastic fiber. FASEB J 1993;7:1208-1218.
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Timpl R, Brown JC. Supramolecular assembly of basement membranes. Bioessays 1996;18:123-132.
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Tilstra DJ, Byers PH. Molecular basis of hereditary disorders of connective tissue. Annu Rev Med 1994;45:149-163. Full articleGo to this article on the publisher's site
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Relevant websites
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Ehrlers-Danlos syndrome: http://www.nlm.nih.gov/medlineplus/ehlersdanlossyndrome.htmlOpen this link in a new window Full articleGo to this article on the publisher's site
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www.ehlers-danlos.org; www.ednf.org Full articleGo to this article on the publisher's site
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Extracellular matrix: http://www.teaching-biomed.man.ac.uk/lawson/ECM.HTMOpen this link in a new window Full articleGo to this article on the publisher's site
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Heparin: www.amhrt.org http://www2.kumc.edu/wichita/meded/cvresource/antithrombotic/heparin/Open this link in a new window Full articleGo to this article on the publisher's site
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Marfan's syndrome: www.marfan.org; www.marfan.org.uk Full articleGo to this article on the publisher's site
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http://www.nlm.nih.gov/medlineplus/marfansyndrome.htmlOpen this link in a new window Full articleGo to this article on the publisher's site
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Mucopolysaccharidoses: http://www.ninds.nih.gov/health_and_medical/pubs/mps.htmOpen this link in a new window Full articleGo to this article on the publisher's site
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http://www.ninds.nih.gov/health_and_medical/disorders/mucopolysaccharidoses.htmOpen this link in a new window Full articleGo to this article on the publisher's site
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Body_ID: P0399
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