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Elastin and the Lung

  • Chapter
Biochemistry of Pulmonary Emphysema

Part of the book series: Current Topics in Rehabilitation ((CURRENT REHAB))

Abstract

Elastin, a rubbery, fibrous connective tissue protein, plays several critical roles in the biomechanics of the lung and its associated vasculature. It is the principal component of the elastic fibre, which is a composite of the amorphous elastic protein and one or more microfibrillar components. Elastic fibres provide resiliency to numerous pulmonary structures including the alveolar wall, the bronchiolar interstitium, the medial layers of arterial and venous elements, lymphatics, and the pleura. The unusual hydrophobic properties of the protein, together with the presence of numerous intermolecular crosslinks, in particular desmosine and isodesmosine, produce a highly insoluble, durable network of polypeptide chains that function as a perfect elastomer in an aqueous environment.

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References

  1. Uitto J., Olsen D.R., Fazio M.J., Rosenbloom J.: Molecular pathology of elastin. In: Tamburro A.M., Davidson J.M. (Eds.) Elastin: Chemical and Biological Aspects. Galatina, Congedo Editore, 1990; 305–330

    Google Scholar 

  2. Ledoux-Corbusier M.: Cutis laxa: congenital form with pulmonary emphysema. An ultrastructural study. J. Cutan. Pathol. 1983; 10: 340–349

    Article  PubMed  CAS  Google Scholar 

  3. Sephel G.C., Byers P.H., Holbrook K.A., Davidson J.M.: Heterogeneity of elastin expression in cutis laxa fibroblast strains. J. Invest. Dermatol. 1989; 93: 147–153

    Article  PubMed  CAS  Google Scholar 

  4. Olsen D.A., Fazio M.J., Shamban A.T., Rosenbloom J., Uitto J.: Cutis laxa-reduced elastin gene expression in skin fibroblast cultures as determined by hybridization with a homologous cDNA and an exon 1-specific oligonucleotide. J. Biol. Chem. 1988; 263: 6465–6468

    PubMed  CAS  Google Scholar 

  5. Soskel N.T., Sandberg L.B.: Pulmonary emphysema. From animal models to human diseases. In: Uitto J., Perejda A., (Eds.) Connective Tissue Disease, New York, Marcel Dekker, 1986; 423–453

    Google Scholar 

  6. Janoff A.: Elastase in tissue injury. Annu. Rev. Med. 1985; 36: 207–216

    Article  PubMed  CAS  Google Scholar 

  7. Bashir M., Indih Z., Yeh H., Abrams W., Ornstein-Goldstein N., Rosenbloom J.C., Fazio M., Uitto J., Mecham R., Rosenbloom J.: Elastin gene structure and mRNA alternative splicing. In: TamburroA.M., DavidsonJ.M. (Eds.)Elastin: ChemicalandBiologicalAspects, Galatina, Congedo Editore 1990; 45–70

    Google Scholar 

  8. Urry D.W.: Entropic elastic processes in protein mechanisms. I. Elastic structure due to an inverse temperature transition and elasticity due to internal chain dynamics. J. Protein. Chem. 1988; 7: 1–34

    CAS  Google Scholar 

  9. Gosline J.M.: Hydrophobic interaction and a model for the elasticity of elastin. Biopolymers 1978; 17: 677–695

    Article  PubMed  CAS  Google Scholar 

  10. Kagan H.M.: Characterization and regulation of lysyl oxidase. In: Mecham R.P., (Ed.) Regulation of Matrix Accumulation New York, Academic Press., 1986; 322–399

    Google Scholar 

  11. Starcher B.C.: Elastin and the lung. Thorax 1986; 41: 577–585

    Article  PubMed  CAS  Google Scholar 

  12. Davidson J.M., Berg R.A.: Posttransational events in collagen biosynthesis. In: Hand A., Oliver C. (Eds.) Meth. Cell Biol. New York, Academic Press 1981; 119–136

    Google Scholar 

  13. Ellis R.J., van der Vies S.M., Hemmingsen S.M.: The molecular chaperone concept. Biochem. Soc. Symp. 1989; 55: 145–153

    PubMed  CAS  Google Scholar 

  14. Davidson J.M., Giro M.G., Sutcliffe M., Zoia O., Quaglino Jr. D., Liu J.M., Perkett E., Meyrick B., Broadley K.M., Russel S., Sephel G.C.: Regulation of elastin synthesis. In: Tamburro A.M., Davidson J.M. (Eds.) Elastin: Chemical and Biological Aspects, Galatina, Congedo Editore 1990; 393–405

    Google Scholar 

  15. De Clerck Y.A., Jones P.A.: The effect of ascorbic acid on the nature an a production of collagen and elastin by rat smooth muscle cells. Biochem J. 1986: 217–225

    Google Scholar 

  16. Dunn D.M., Franzblau C.: Effects of ascorbate on insoluble elastin accumulation and crosslink formation in rabbit pulmonary artery smooth muscle cell cultures. Biochemistry 1983; 21: 4195–4202

    Article  Google Scholar 

  17. Indik Z., Yeh H., Ornstein-Goldstein N., Sheppard P., Anderson N., Rosenbloom J.C., Peltoman L., Rosenbloom J.: Alternative splicing of human elastin mRNA indicated by sequence analysis of cloned genomic and complementary DNA. Proc. Natl. Acad. Sci. USA 1987; 84: 5680–5684

    Article  PubMed  CAS  Google Scholar 

  18. Fazio M.J., Mattei M.G., Chu M.L., Black D., Solomon E., Davidson J.M., Uitto J.: Human elastin gene: Chromosomal mapping to locus 7g11.2. Am. J. Hum. Genet. 1991; 48: 696–703

    PubMed  CAS  Google Scholar 

  19. Raju K., Anwar R.A.: Primary structures of bovine elastin a,b, and c deduced from the sequences of cDNA clones. J. Biol. Chem. 1987; 262: 5755–5762

    PubMed  CAS  Google Scholar 

  20. Pollock J., Baule V.J., Rich C.B., Ginsburg C.D., Curtis S., Foster J.A.: Chick tropoelastin isoforms. From the gene to the extracellular matrix. J. Biol. Chem. 1990; 265: 3697–3702

    PubMed  CAS  Google Scholar 

  21. Wreen D.S., Parks W.C., Whitehouse L.A., Crouch E.C., Kinich U., Rosenbloom J., Mecham R.P.: Identification of multiple tropoelastins secreted by bovine cells. J. Biol. Chem. 1987; 262: 2244–2249

    Google Scholar 

  22. Fazio M.J., Kahari V.M., Bashir M.M., Saitla B., Rosenbloom J., Uitto J.: Regulation of elastin gene expression: evidence for functional promoter activity in the 5-flanking region of the human gene. J. Invest. Dermatol. 1990; 94: 181–196

    Article  Google Scholar 

  23. Cleary E.G.: The microfibrillar component of the elastic fibers. Morphology and Biochemistry. In: Uitto J., Perejda A. (Eds.) Connective Tissue Disease. New York, Marcel Dekker 1986; 55–81

    Google Scholar 

  24. Ross R., Bornstein P.: Elastic fibers in the body. Sci. Am. 1971; 224: 44–52

    PubMed  CAS  Google Scholar 

  25. Sakai L.Y., Keene D.R., Engvall E.: Fibrillin, a new 350kD glycoprotein, is a component of extracellular microfibrils. J. Cell. Biol. 1986; 103: 2499–2509

    Article  PubMed  CAS  Google Scholar 

  26. Maddox B.K., Sakai L.Y., Keene D.R., Glanville R.W.: Connective tissue microfibrils. Isolation and characterization of three large pepsin-resistant domains of fibrillin. J. Biol. Chem. 1989; 264: 21381–21385

    PubMed  CAS  Google Scholar 

  27. Gibson M.A., Kumaratilake J.S., Cleary E.G.: The protein components of the 12-nanometer microfibrils of elastic and nonelastic tissue. J. Biol. Chem. 1989; 264: 4590–4598

    PubMed  CAS  Google Scholar 

  28. Hollister D.W., Godfrey M., Sakai L.Y., Pyeritz R.E.: Immunohistologic abnormalities of the microfibrillar-fiber system in the Marfan syndrome. N. Engl. J. Med. 1990; 32: 152–159

    Article  Google Scholar 

  29. Hinek A., Wrenn D.S., Mecham R.P., Barondes S.H.: The elastin receptor, a galactoside-binding protein. Science 1988; 239: 1539–1541

    Article  PubMed  CAS  Google Scholar 

  30. Mecham R P, Hinek A., Entwistle R., Wrenn D.S., Griffin G.L., Senior R.M.: Elastin binds to a multifunctional 97-Kilodalton peripheral membrane protein. Biochemistry 1989; 28: 3716–3722

    Article  PubMed  CAS  Google Scholar 

  31. Mecham R.P., Hinek A., Griffin G.L., Senior R.M., Liotta L.A.: The elastin receptor shows structural and functional similarities to the 67-kDa tumor cell laminin receptor. J. Biol. Chem. 1989; 264: 16652–16657

    PubMed  CAS  Google Scholar 

  32. Blood C.H., Sasse J., Brodt P., Zetter B.R.: Identification of a tumor cell receptor for VGVAPG, an elastin-derived chemotactic peptide. J. Cell. Biol. 1988; 107: 1987–1993

    Article  PubMed  CAS  Google Scholar 

  33. Netland P.A., Zetter B.R.: Melanoma cell adhesion to defined extracellular matrix components. Biochem. Biophys. Res. Commun. 1986; 139: 515–522

    Article  PubMed  CAS  Google Scholar 

  34. Hunninghake G.W., Davidson J.M., Rennard S.I., Szapiel S., Gadek J., Crystal R.G.: Mechanisms of pulmonary emphysema. Attraction of macrophage precursors to sites of disease activity by elastin fragments. Science, 1981; 212: 925–927

    Article  PubMed  CAS  Google Scholar 

  35. Tobias J.W., Bern M.M., Netland P.A., Zetter B.R.: Monocyte adhesion to subendothelial components. Blood 1987; 69: 1265–1268

    PubMed  CAS  Google Scholar 

  36. Senior R.M., Hinek A., Griffin G.L., Pipoly D.J., Crouch E.C., Mecham R.P.: Neutrophils show chemotaxis to type IV collagen and its 7S domain and contain a 67 kD type IV collagen binding protein with lectin properties. Am. J. Respir. Cell Mol. Biol. 1989; 1: 479–487

    Article  PubMed  CAS  Google Scholar 

  37. Cerra R.F., Haywood Reed P.L., Barondes S.H.: Endogenous mammalian lectin localized extracellularly in lung elastic fibers. J. Cell. Biol. 1984; 98: 1580–1589

    Article  PubMed  CAS  Google Scholar 

  38. Powell J.T.: Evidence against lung galaptin being important to the synthesis or organization of the elastic fibril. Biochem. J. 1988; 252: 447–452

    PubMed  CAS  Google Scholar 

  39. Hornebeck W., Tixier J.M., Robert L.: Inducible adhesion of mesenchymal cells to elastic fibers. Proc. Natl. Acad. Sci. USA, 1986; 83: 5517–5520

    Article  PubMed  CAS  Google Scholar 

  40. Robert L. Jacob M.P., Fulop T., Timar J., Hornebeck W.: Elastonectin in the elastin receptor. Pathol. Biol. (Paris) 1989; 37: 736–741

    CAS  Google Scholar 

  41. Clark J.M., Glagov S.: Transmural organization of the arterial media. The lamellar unit revisited. Arteriosclerosis 1985; 5: 19–34

    Article  PubMed  CAS  Google Scholar 

  42. Shibahara S., Davidson J.M., Smith K., Crystal R.O.: Modulation of elastin synthesis and mRNA activity in developing sheep lung. Biochemistry 1981; 20: 6577–6584

    Article  PubMed  CAS  Google Scholar 

  43. Myers B., DubickM., Last J.A., Rucker R.B.: Elastin synthesis during perinatal lung development in the rat. Biochim Biophys. Acta 1983; 761: 17–22

    CAS  Google Scholar 

  44. Lefevre M., Rucker R.B.: Aorta elastin turnover in normal and hypercholesterolemic Japanese quail. Biochim. Biophys. Acta 1980; 630: 519–529

    Article  PubMed  CAS  Google Scholar 

  45. Christie R.: The elastic properties of the emphysematous lung and their clinical significance. J. Clin. Invest. 1934; 13: 295–297

    Article  PubMed  CAS  Google Scholar 

  46. Laurell C.N., Eriksson S.: The electrophoretic al-globulin pattern of serum in al-antitrypsin deficiency. Scand. J. Clin. Lab. Invest. 1963; 15: 132–140

    CAS  Google Scholar 

  47. Kuhn C., Senior R.M.: The role of elastases in the development of emphysema. Lung. 1978; 155: 185–197

    Article  PubMed  Google Scholar 

  48. Pierce J.A., Hocott J.B., Ebert R.V.: The collagen and elastin content of the lung in emphysema. Ann. Intern. Med. 1961; 55: 210–222

    Article  PubMed  CAS  Google Scholar 

  49. Meyrick B., Reid L.M.:In: Hodson W.A. (Ed.) Development of the Lung. New York, Marcel Dekker, 1977; 135–214

    Google Scholar 

  50. Quaglino Jr D., Nanney L.B., Kennedy R., Davidson J.M.: Localized effects of transforming growth factor-13 on extracellular matrix gene expression during wound healing. I. Excisional wound model. Lab. Invest. 1990; 63: 307–319

    PubMed  CAS  Google Scholar 

  51. Davidson J.M., Giro M.G.: Repair of elastic tissue. In: Cohen I.K., Digelmann R., Lindblad W. (Eds.) Wound Repair, 3rd ed. New York, Saunders, 1991

    Google Scholar 

  52. Perkett E.A., Brigham L.K., Meyrick B.: Continuous air embolization into sheep causes sustained pulmonary hypertension and increased vascular reactivity. Am. J. Path. 1988; 123: 444–454

    Google Scholar 

  53. Mecham R.P., Withehouse L.A., Wrenn D.S., Parks W.C., Griffin G.L., Senior R.M., Crouch E.C., Stenmark K.R., Voelkel N.F.: Smooth muscle-mediated connective tissue remodeling in pulmonary hypertension. Science 1987; 237: 423–426

    Article  PubMed  CAS  Google Scholar 

  54. Prosser I.W.; Stenmark K.R., Suthar M., Crouch E.C., Mecham R.P., Parks W.C.: Regional heterogeneity of elastin and collagen gene expression in intralobar arteries in response to hypoxic pulmonary hypertension as demonstrated by in situ hybridization. Am. J. Path. 1989;135:10731087

    Google Scholar 

  55. Liu J.M., Davidson J.M.: The elastogenic effect of recombinant transforming growth factor-13 on porcine aortic smooth muscle cells. Biochem. Biophys. Res. Commun. 1988; 154: 895–901

    Article  PubMed  CAS  Google Scholar 

  56. Roberts A.B., Flanders K.C., Kondaiah P., Thompson N.I., Van Obberghen-Schilling E., Wakefield L., Rossi P., de Crombrugghe B., Heine U., Sporn M.B.: Transforming growth factor-13, biochemistry and roles in embryogenesis, tissue repair and remodeling, and carcinogenesis. Rec. Prog. Horm. Res. 1988; 44: 157–197

    PubMed  CAS  Google Scholar 

  57. Yamauchi K., Martinet Y., Basset P., Fells G.S., Crystal R.G.: High levels of transforming growth factor-(3 are present in the epithelial lining fluid of the normal human respiratory tract. Am. Rev. Resp. Dis. 1988; 137: 1360–1363

    Article  PubMed  CAS  Google Scholar 

  58. Perkett E.A., Lyons R.M., Moses H.L., Brigham K.L., Meyrick B.: Transforming growth factor-13 activity in sheep lung lymph during development of pulmonary hypertension. J. Clin. Invest., 1990, 86, 1459–1464

    Article  PubMed  CAS  Google Scholar 

  59. Perkett E.A.: Personal communication

    Google Scholar 

  60. Foster J., Rich C.B., Florini J.R.: Insulin-like growth factor, somatomedin C, induces the synthesis of elastin in aortic tissue. Coll. Rel. Res. 1987; 7: 161–169

    Article  CAS  Google Scholar 

  61. Badesch D.B., Lee P.D., Parks W.C., Stenmark K.R.: Insulin-like growth factor I stimulates elastin synthesis by bovine pulmonary arterial smooth muscle cells. Biochem Biophys. Res. Commun. 1989; 160: 382–397

    CAS  Google Scholar 

  62. Foster J.A., Miller M.L., Benedict M.R., Richmann R.A., Rich C.B.: Evidence for insulin-like growth factor regulation of chick aortic elastogenesis. Matrix 1989; 9: 328–335

    Article  PubMed  CAS  Google Scholar 

  63. Rom W.N., Basset P., Fells G.A., Nukiwa T., Trapnell B.C., Crystal R.G.: Alveolar macrophages release an insulin-like growth factor-I-type molecule. J. Clin. Invest. 1988; 82: 1685–1693

    Article  PubMed  CAS  Google Scholar 

  64. Trackman P.C., Pratt A.M., Wolanski A., Tang S.S., Offner G.D., Troxler R.F., Kagan H.M.: Cloning of rat aorta lysyl oxidase cDNA: Complete codons and predicted amino acid sequence. Biochemistry 1990; 29: 4863–4870

    Article  PubMed  CAS  Google Scholar 

  65. King G.S., Starcher B.C., Kuhn C.T.: The measurement of elastin turnover by the radioimmunoassay of urinary desmosine excretion. Bull. Eur. Physiopathol. Respir. 1980; 16 (suppl): 61–64

    PubMed  CAS  Google Scholar 

  66. Pelham F., Wewers M., Crystal R., Buist A.S., Janoff A.: Urinary excretion of desmosine (elastin cross-links) in subjects with PiZZ al-antitrypsin deficiency, a phenotype associated with hereditary disposition to pulmonary emphysema. Am. Rev. Respir. Dis. 1985; 132: 821–823

    PubMed  CAS  Google Scholar 

  67. Schriver E.E., Bernard G.R., Swindell B.B., Sutcliffe M.S., Davidson J.M.: Elastin fragment levels in human plasma, urine, and bronchoalveolar lavage fluid. (BALF) Chest 1989; 96: 1535

    Google Scholar 

  68. Perkett E.A., Davidson J.M., Curtis-Atchley P., Sutcliffe M.C., Brigham K.L., Meyrick B.: Lung lymph elastin concentration is increased in sheep with pulmonary hypertension secondary to air embolization. Am. Rev. Respir. Dis. 1989; 139: A174

    Google Scholar 

  69. Gadek J.W., Hunninghake G.W., Fells G.A., Zimmerman R.L., Keogh B.A., Crystal R.G.: Evaluation of the protease-antiprotease theory of human destructive lung disease. Bull. Eur. Physiopath. Resp. 1980; 16 (Suppl): 27–40

    Google Scholar 

  70. Davidson J.M., Zoia O., Nair R., Meyrick B., Perkett E.A.: unpublished studies

    Google Scholar 

  71. Davidson J.M., Giro M.G.: Control of elastin synthesis: Molecular and cellular aspects. In: Mecham R.P. (Ed.) Regulation of Matrix Accumulation. New York, Academic Press, 1986;177216

    Google Scholar 

  72. Davidson J.M.: Biochemistry and turnover of lung interstitium. Eur. Resp. J. 1990; 3: 1048–1068

    CAS  Google Scholar 

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Authors and Affiliations

  1. Research Service, Department of Veterans Affairs Medical Center and Department of Pathology, Vanderbilt University School of Medicine, Nashville, Tennessee, USA

    J. M. Davidson

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  1. J. M. Davidson
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Editors and Affiliations

  1. Institute of Phthisiology and Respiratory Diseases, IRCCS San Matteo, University of Pavia, Italy

    C. Grassi  & M. Luisetti  & 

  2. Department of Biochemistry, University of Georgia, Athens, GA, USA

    J. Travis

  3. Postgraduate School of Respiratory Diseases, University of Pavia, Italy

    L. Casali

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© 1992 Springer-Verlag London

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Davidson, J.M. (1992). Elastin and the Lung. In: Grassi, C., Travis, J., Casali, L., Luisetti, M. (eds) Biochemistry of Pulmonary Emphysema. Current Topics in Rehabilitation. Springer, London. https://doi.org/10.1007/978-1-4471-3771-9_2

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  • DOI: https://doi.org/10.1007/978-1-4471-3771-9_2

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