The Morphogenesis of Elastic Fibers
Elastic fibers have been shown to contain two proteins, insoluble elastin and the elastic fiber microfibril, a glycoprotein. The microfibril has been suggested to play a morphogenetic role in determining the presumptive shape and direction of the forming elastic fiber. The principal alteration seen in individuals with the disease Pseudoxanthoma Elasticum is in insoluble elastin which loses its amorphous appearance and affinity for anionic stains, and takes on a finely granular appearance and shows increased affinity for cationic stains. Normal elastic fiber microfibrils are sometimes associated with this material; although, in general, these structures are not present in the elastic fibers that are markedly altered in this disease.
KeywordsElastic Fiber Polar Amino Acid Granular Appearance Pseudoxanthoma Elasticum Digital Flexor Tendon
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- 2.Ross R., Bornstein, P., Studies of the components of the elastic fiber, Chemistry and Molecular Biology of the Intercellular Matrix. Edited by Balazs. Academic Press, London, 1970, p. 641–656.Google Scholar
- 3.Ross, Russell, The Elastic Fiber, A Review, J. Histochem & Cytochem, 21:199–208.Google Scholar
- 9.Ross, R. and Bornstein, P., Elastic fibers in the body. Sci Am, Vol. 224, No. 6, pp. 44–52.Google Scholar
- 10.Altman, L.K., Fialkow, P.J., Parker, F. and Sagebiel, R.W., Pseudoxanthoma Elastlcum, Arch Int. Med, 1974, Vol. 134.Google Scholar