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Structure and variation of human α1–antitrypsin

Nature volume 298, pages 329–334 (1982)Cite this article

Abstract

The sequence of α1–antitrypsin is in keeping with its role as a tissue scavenger of leukocyte elastase. Two abnormal variants commonly present in Europeans cause a deficiency that predisposes them to a progressive loss of lung elasticity. The nature of the reactive centre helps explain why cigarette smoking greatly accelerates the onset and severity of this degenerative process to give the disease emphysema.

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References

  1. Laurell, C.-B. & Eriksson, S. Scand. J. clin. Lab. Invest. 15, 132–140 (1963).

    Article  CAS  Google Scholar 

  2. Eriksson, S. Acta med. scand. 175, 197–205 (1964).

    Article  CAS  Google Scholar 

  3. Sharp, H. L. Hosp. Pract. 6, 83–96 (1971).

    Article  Google Scholar 

  4. Fagerhol, M. K. Postgrad. med. J. 52 Suppl. 2, 73–83 (1976).

    PubMed  Google Scholar 

  5. Carrell, R. W. & Owen, M. C. Essays med. Biochem. 4, 83–119 (1979).

    CAS  Google Scholar 

  6. Lancet i, 743–745 (1980).

  7. Gadek, J. E. et al. Bull. Eur. Physiopath. Resp. 16, suppl., 27–40 (1980).

    Google Scholar 

  8. Larsson, C. Acta med. scand. 204, 345–351 (1978).

    Article  CAS  Google Scholar 

  9. Mega, T., Lujan, E. & Yoshida, A. J. biol. Chem. 255, 4053–4056 (1980).

    CAS  PubMed  Google Scholar 

  10. Carrell, R. W. et al. FEBS Lett. 135, 301–303 (1981).

    Article  CAS  Google Scholar 

  11. Kurachi, K. et al. Proc. natn. Acad. Sci. U.S.A. 78, 6826–6830 (1981).

    Article  ADS  CAS  Google Scholar 

  12. Carlson, J. & Stenflo, J. FEBS Lett. 130, 297–300 (1981).

    Article  CAS  Google Scholar 

  13. Boswell, D. R., Owen, M. C., Brennan, S. O., Carrell, R. W. & McLachlan, A. D. in Protides of the Biological Fluids: 27th Colloqu. 28, 129–132 (1980).

    Google Scholar 

  14. Johnson, D. & Travis, J. J. biol. Chem. 253, 7142–7144 (1978).

    CAS  PubMed  Google Scholar 

  15. Kraut, J. A. Rev. Biochem. 46, 331–358 (1977).

    Article  CAS  Google Scholar 

  16. Jirgensons, B. Biochim. biophys. Acta 493, 352–358 (1977).

    Article  CAS  Google Scholar 

  17. Saklatvala, J., Wood, G. C. & White, D. D. Biochem. J. 157, 339–351 (1976).

    Article  CAS  Google Scholar 

  18. Owen, M. C. thesis, Univ. Otago, Dunedin (1979).

  19. Peterson, T. E., Dudek-Wojciechowska, G., Sottrup-Jensen, L. & Magnusson, S. in The Physiological Inhibitors of Blood Coagulation and Fibrinolysis (eds Collen, D. et al.). 43–54 (Elsevier, Amsterdam, 1979).

    Google Scholar 

  20. Laurell, C.-B. in The Chemistry and Physiology of the Human Plasma Proteins (ed. Bing, D. H.) 329–338 (Pergamon, New York, 1979).

    Google Scholar 

  21. Fagerhol, M. K. Ser. Haemat. 1, Suppl. 1, 153–161 (1968).

    CAS  Google Scholar 

  22. Allen, R. C., Harley, R. H. & Talamo, R. C. Am. J. clin. Path. 62, 732–739 (1974).

    Article  CAS  Google Scholar 

  23. Vaughan, L. & Carrell, R. W. Biochem. Int. 2, 461–467 (1981).

    CAS  Google Scholar 

  24. Martodam, R. R. & Liener, I. E. Biochim. biophys. Acta 667, 328–340 (1981).

    Article  CAS  Google Scholar 

  25. Kress, L. F., Kurecki, T., Chan, S. K. & Laskowski, Srn. M. J. biol. Chem. 254, 5317–5320 (1979).

    CAS  PubMed  Google Scholar 

  26. James, H. L. & Cohen, A. B. J. clin. Invest. 62, 1344–1353 (1978).

    Article  CAS  Google Scholar 

  27. Carrell, R. W., Boswell, D. R., Brennan, S. O. & Owen, M. C. Biochem. Biophys. Res. Commun. 93, 399–402 (1980).

    Article  CAS  Google Scholar 

  28. Morii, M., Odani, S. & Ikenaka, T. J. Biochem. 86, 915–921 (1979).

    Article  CAS  Google Scholar 

  29. Cohen, A. B. J. biol. Chem. 248, 7055–7059 (1973).

    CAS  PubMed  Google Scholar 

  30. Carrell, R. W., Owen, M. C., Brennan, S. O. & Vaughan, L. Biochem. biophys. Res. Commun. 91, 1032–1037 (1979).

    Article  CAS  Google Scholar 

  31. Hunt, L. T. & Dayhoff, M. O. Biochem. biophys. Res. Commun. 95, 864–871 (1980).

    Article  CAS  Google Scholar 

  32. Leicht, M. et al. Nature 297, 655–659 (1982).

    Article  ADS  CAS  Google Scholar 

  33. Beatty, K., Bieth, J. & Travis, J. J. biol. Chem. 255, 3931–3934 (1980).

    CAS  PubMed  Google Scholar 

  34. Gauthier, F., Fryksmark, U., Ohlsson, L. & Bieth, J. G. Biochim. biophys. Acta 700, 178–183 (1982).

    Article  CAS  Google Scholar 

  35. Travis, J., Garner, D. & Bowen, J. Biochemistry 17, 5647–5651 (1978).

    Article  CAS  Google Scholar 

  36. Jeppsson, J.-O., Laurell, C.-B., Nosslin, B. & Cox, D. W. Clin. Sci. Molec. Med. 5, 103–107 (1978).

    Google Scholar 

  37. Jeppsson, J.-O., Laurell, C.-B. & Fagerhol, M. K. Eur. J. Biochem. 83, 143–153 (1978).

    Article  CAS  Google Scholar 

  38. Vaughan, L., Lorier, M. A. & Carrell, R. W. Biochim. biophys. Acta 701, 339–345 (1982).

    Article  CAS  Google Scholar 

  39. Balldin, G., Laurell, C.-B. & Ohlsson, K. Hoppe-Seylers Z. physiol. Chem. 359, 699–708 (1978).

    CAS  PubMed  Google Scholar 

  40. Senior, R. et al. Am. Rev. resp. Dis. 116, 469–475 (1977).

    Article  CAS  Google Scholar 

  41. Gadek, J. E., Fells, G. A., Zimmerman, R. L., Rennard, S. I. & Crystal, R. G. J. clin. Invest. 68, 889–898 (1981).

    Article  CAS  Google Scholar 

  42. Nakajima, K., Powers, J. C., Ashe, B. M. & Zimmerman, M. J. biol. Chem. 254, 4027–4032 (1979).

    CAS  PubMed  Google Scholar 

  43. Shechter, Y., Burstein, Y. & Gertler, A. Biochemistry 16, 992–997 (1977).

    Article  CAS  Google Scholar 

  44. Beatty, K., Robertie, P., Senior, R. & Travis, J. J. Lab. clin. Med. (in the press).

  45. Carp, H. & Janoff, A. Am. Rev. resp. Dis. 118, 617–621 (1978).

    CAS  PubMed  Google Scholar 

  46. Matheson, N. R., Wong, P. S. & Travis, J. Biochem. biophys. Res. Commun. 88, 402–409 (1979).

    Article  CAS  Google Scholar 

  47. Baglioni, M. in The Cell Biology of Inflammation (ed. Weissman, G.) 161–187 (Elsevier, Amsterdam, 1980).

    Google Scholar 

  48. Gadek, J. E., Fells, G. A. & Crystal, R. G. Science 206, 1315–1316 (1979).

    Article  ADS  CAS  Google Scholar 

  49. Laurell, C.-B. & Jeppsson, J.-O. in The Plasma Proteins. Structure, Function and Genetic Control Vol. 1, 2nd edn (ed. Putnam, F. W.) 229–264 (Academic, New York, 1975).

    Google Scholar 

  50. Owen, M. C. & Carrell, R. W. Br. med. J. i, 130–131 (1976).

    Google Scholar 

  51. Jeppsson, J.-O. FEBS Lett. 65, 195–197 (1976).

    Article  CAS  Google Scholar 

  52. Yoshida, A., Lieberman, J., Gaidulis, L. & Ewing, C. Proc. natn. Acad. Sci. U.S.A. 73, 1324–1328 (1976).

    Article  ADS  CAS  Google Scholar 

  53. Laurell, C.-B. & Sveger, T. Am. J. hum. Genet. 27, 213–217 (1975).

    CAS  PubMed  PubMed Central  Google Scholar 

  54. Fagerhol, M. K. & Cox, D. W. Adv. hum. Genet. 11, 1–62 (1981).

    CAS  PubMed  Google Scholar 

  55. Blenkinsopp, W. K. & Haffenden, G. P. J. clin. Path. 30, 132–137 (1977).

    Article  CAS  Google Scholar 

  56. Jeppsson, J.-O., Larsson, C. & Eriksson, S. New Engl. J. Med. 293, 576–579 (1975).

    Article  CAS  Google Scholar 

  57. Hercz, A., Katona, E., Cutz, E., Wilson, J. R. & Barton, M. Science 201, 1229–1232 (1978).

    Article  ADS  CAS  Google Scholar 

  58. Sveger, T. New Engl. J. Med. 294, 1316–1321 (1976).

    Article  CAS  Google Scholar 

  59. Lieberman, J. Chest 70, 62–67 (1976).

    Article  CAS  Google Scholar 

  60. Janoff, A., Carp, H., Lee, D. K. & Drew, R. T. Science 206, 1313–1316 (1979).

    Article  ADS  CAS  Google Scholar 

  61. Carp, H. & Janoff, A. J. clin. Invest. 63, 793–797 (1979).

    Article  CAS  Google Scholar 

  62. Janus, E. D. & Carrell, R. W. N. Z. med. J. 81, 461–467 (1975).

    CAS  PubMed  Google Scholar 

  63. Larsson, C., Ericksson, S. & Dirkson, H. Br. med. J. ii, 922–925 (1977).

    Article  Google Scholar 

  64. de Hamel, F. A. & Carrell, R. W. N. Z. med. J. 94, 407–410 (1981).

    CAS  PubMed  Google Scholar 

  65. Yoshida, A., Ewing, C., Wessels, M., Lieberman, J. & Gaidulis, L. Am. J. hum. Genet. 29, 233–239 (1977).

    CAS  PubMed  PubMed Central  Google Scholar 

  66. Lieberman, J. Chest. 64, 579–584 (1973).

    Article  CAS  Google Scholar 

  67. Bell, O. F. & Carrell, R. W. Nature 243, 410–411 (1973).

    Article  ADS  CAS  Google Scholar 

  68. McReynolds, L. et al. Nature 273, 723–728 (1978).

    Article  ADS  CAS  Google Scholar 

  69. Moo-Penn, W. F. et al. J. biol. Chem. 251, 7557–7562 (1976).

    CAS  PubMed  Google Scholar 

  70. Perutz, M. F., Fogg, J. H. & Fox, J. A. J. molec. Biol. 138, 669–670 (1980).

    Article  CAS  Google Scholar 

  71. Lingappa, V. R., Lingappa, J. R. & Blobel, G. Nature 281, 117–121 (1979).

    Article  ADS  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Molecular Pathology Laboratory, Pathology Department (University of Otago), Christchurch Hospital, Christchurch, New Zealand

    Robin W. Carrell, Stephen O. Brennan, Maurice C. Owen, Lloyd Vaughan & D. Ross Boswell

  2. Department of Clinical Chemistry, University of Lund, Malmö General Hospital, S-214 01, Malmö, Sweden

    Jan-Olof Jeppsson & Carl-Bertil Laurell

Authors
  1. Robin W. Carrell
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  6. Lloyd Vaughan
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  7. D. Ross Boswell
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Carrell, R., Jeppsson, JO., Laurell, CB. et al. Structure and variation of human α1–antitrypsin. Nature 298, 329–334 (1982). https://doi.org/10.1038/298329a0

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