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Heritable Connective Tissue Disorders

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Rheumaderm

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 455))

Abstract

The extracellular matrix of connective tissue of the skin consists of a large number of distinct matrix macromolecules which play a critical role in providing physiologic properties to normal skin. Within the dermis, collagen and elastic fibers are the two major fibrillar components of connective tissue, and they exist in a fibrous interwoven mesh- work structure associated with other extracellular macromolecules of the dermis, such as proteoglycan/glycosaminoglycan complexes, and a number of non-collagenous glycoproteins, including fibronectin and fibrillins (Tables 1 and 2). Encased in the fibrillar mesh- work are cells that interact with the extracellular matrix components through cell-matrix receptors, such as integrins.

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Selected Reviews

  • Uitto, J., and Fazio, M. Biology and Pathophysiology of the Extracellular Matrix in Dermis. In: Pathophysiology of Dermatologic Diseases (Soter, N.A., and Baden, H.P., eds), 2nd Edition, McGraw-Hill, New York, pp.349–367, 1990.

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  • Uitto, J., Fazio, M.J., and Olsen, D.R. Heritable Disorders of Connective Tissue. In: Genetic Disorders of the Skin (Alper, J.C., ed), Mosby-Year Book, Inc., St. Louis, MO, pp. 287–319, 1991.

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  • Uitto, J., McGrath, J., and Bernstein, E. The Dermis. In: Cutaneous Medicine and Surgery: An Integrated Program in Dermatology (K.A. Arndt, P.E. LeBoit, J.K. Robinson, and B.U. Wintroub, eds), W.B. Saunders Company, Philadelphia, PA, vol. 1, pp. 857–881, 1996.

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  • Rosenbloom, J. The Elastic Fiber in Health and Disease. Progress in Dermatology, vol. 30(4), pp. 1–15, 1996.

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Correspondence to Jouni Uitto M.D., Ph.D. .

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© 1999 Springer Science+Business Media New York

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Uitto, J. (1999). Heritable Connective Tissue Disorders. In: Mallia, C., Uitto, J. (eds) Rheumaderm. Advances in Experimental Medicine and Biology, vol 455. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-4857-7_3

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  • DOI: https://doi.org/10.1007/978-1-4615-4857-7_3

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-7203-5

  • Online ISBN: 978-1-4615-4857-7

  • eBook Packages: Springer Book Archive

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