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The FASEB Journal, Vol 7, 1208-1218, Copyright © 1993 by The Federation of American Societies for Experimental Biology
REVIEWS |
J Rosenbloom, WR Abrams and R Mecham
Department of Anatomy and Histology, School of Dental Medicine, University of Pennsylvania, Philadelphia 19104.
The elastic properties of many tissues such as the lung, dermis, and large blood vessels are due to the presence of elastic fibers in the extracellular space. These fibers have been shown by biochemical and ultrastructural analysis to be composed of two distinct components, a more abundant amorphous component and a 10-12 nm microfibrillar component, which is located primarily around the periphery of the amorphous component. The protein elastin makes up the highly insoluble amorphous component and is responsible for the elastic properties. Elastin is found throughout the vertebrate kingdom and possesses an unusual chemical composition rich in glycine, proline, and hydrophobic amino acids, consonant with its characteristic physical properties. The 72-kDa biosynthetic precursor, tropoelastin, is secreted into the extracellular space where it becomes highly cross-linked into a rubber- like network through the activity of the copper-requiring enzyme lysyl oxidase. Analysis of the elastin gene has demonstrated that hydrophobic and cross-linking domains are encoded in separate exons and that there is significant alternative splicing, resulting in multiple isoforms of tropoelastin. The elastin gene promoter contains many potential binding sites for various modulating factors indicative of a complex pattern of transcriptional regulation. The microfibrils contain several proteins, including fibrillin, and probably act as an organizing scaffold in the formation of the elastin network. There appears to be a fibrillin gene family in which each protein contains multiple repeats of a motif previously found in epidermal growth factor and a second motif observed in transforming growth factor beta 1-binding protein. Mutations in the fibrillin gene located on human chromosome 15 have been strongly implicated as the cause of the Marfan syndrome.
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G. Faury, S. Garnier, A. S. Weiss, J. Wallach, T. Fulop Jr, M.-P. Jacob, R. P. Mecham, L. Robert, and J. Verdetti Action of Tropoelastin and Synthetic Elastin Sequences on Vascular Tone and on Free Ca2+ Level in Human Vascular Endothelial Cells Circ. Res., February 23, 1998; 82(3): 328 - 336. [Abstract] [Full Text] [PDF]
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S. Q. Liu Regression of hypoxic hypertension-induced changes in the elastic laminae of rat pulmonary arteries J Appl Physiol, May 1, 1997; 82(5): 1677 - 1684. [Abstract] [Full Text] [PDF]
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S. Q. Liu Alterations in structure of elastic laminae of rat pulmonary arteries in hypoxic hypertension J Appl Physiol, November 1, 1996; 81(5): 2147 - 2155. [Abstract] [Full Text] [PDF]
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P. W. Park, J. Rosenbloom, W. R. Abrams, J. Rosenbloom, and R. P. Mecham Molecular Cloning and Expression of the Gene for Elastin-binding Protein (ebpS) in Staphylococcus aureus J. Biol. Chem., June 28, 1996; 271(26): 15803 - 15809. [Abstract] [Full Text] [PDF]
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D.J. Johnson, P. Robson, Y. Hew, and F.W. Keeley Decreased Elastin Synthesis in Normal Development and in Long-term Aortic Organ and Cell Cultures Is Related to Rapid and Selective Destabilization of mRNA for Elastin Circ. Res., December 1, 1995; 77(6): 1107 - 1113. [Abstract] [Full Text]
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J. Taipale, J. Lohi, J. Saarinen, P. T. Kovanen, and J. Keski-Oja Human Mast Cell Chymase and Leukocyte Elastase Release Latent Transforming Growth Factor-beta1 from the Extracellular Matrix of Cultured Human Epithelial and Endothelial Cells J. Biol. Chem., March 3, 1995; 270(9): 4689 - 4696. [Abstract] [Full Text] [PDF]
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S. A. Jensen, B. Vrhovski, and A. S. Weiss Domain 26 of Tropoelastin Plays a Dominant Role in Association by Coacervation J. Biol. Chem., September 8, 2000; 275(37): 28449 - 28454. [Abstract] [Full Text] [PDF]
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