Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Letter
  • Published:

Molecular Pathology of Human Haemoglobin: Stereochemical Interpretation of Abnormal Oxygen Affinities

Nature volume 232, pages 408–413 (1971)Cite this article

Abstract

WHEN the atomic model of oxyhaemoglobin had just been completed, we showed that the haemoglobin molecule is insensitive to replacements of most amino-acid residues on its surface but extremely sensitive to quite small alterations of internal non-polar contacts. And replacements near the haems and at the contacts between α and β subunits affect the respiratory function1.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Similar content being viewed by others

References

  1. Perutz, M. F., and Lehmann, H., Nature, 219, 902 (1968).

    Article  ADS  CAS  Google Scholar 

  2. Muirhead, H., and Greer, J., Nature, 228, 516 (1970).

    Article  ADS  CAS  Google Scholar 

  3. Bolton, W., and Perutz, M. F., Nature, 228, 551 (1970).

    Article  ADS  CAS  Google Scholar 

  4. Perutz, M. F., Nature, 228, 726 (1970).

    Article  ADS  CAS  Google Scholar 

  5. Buse, G., Braig, S., and Braunitzer, G., Hoppe Seyl. Z., 349, (1969).

  6. Rossi Fanelli, A., Antonini, E., and Povoledo, D., Symposium on Protein Structure (edit. by Neuberger, A.), 144 (Methuen, London, 1958).

    Google Scholar 

  7. Imai, K., Morimoto, H., Kotani, M., Watari, H., Hirata, W., and Kuroda, M., Biochim. Biophys. Acta, 200, 189 (1970).

    Article  CAS  Google Scholar 

  8. Dacie, J. V., Shinton, N. K., Gaffney, P. J., Carrell, R. W., and Lehmann, H., Nature, 216, 663 (1967).

    Article  ADS  CAS  Google Scholar 

  9. Grimes, A. J., Meisler, A., and Dacie, J. V., Brit. J. Haematol., 10, 281 (1964).

    Article  CAS  Google Scholar 

  10. Muller, C. J., and Kingma, S., Biochim. Biophys. Acta, 50, 595 (1961).

    Article  CAS  Google Scholar 

  11. Winterhalter, K. H., Anderson, N. M., Amiconi, G., Antonini, E., and Brunori, M., Europ. J. Biochem., 11, 435 (1969).

    Article  CAS  Google Scholar 

  12. Hitzig, W. H., Frick, P. C., Betke, K., and Huisman, T. H. J., Helv. Paediat. Acta, 15, 499 (1960).

    CAS  PubMed  Google Scholar 

  13. Steadman, J. H., Yates, A., and Huehns, E. R., Brit. J. Haematol., 18, 435 (1970).

    Article  CAS  Google Scholar 

  14. Cathie, I. A. B., Great Ormond Street J., 2, 43 (1952).

    Google Scholar 

  15. Hollender, A., Lorkin, P. A., Lehmann, H., and Svensson, B., Nature, 222, 953 (1969).

    Article  ADS  CAS  Google Scholar 

  16. Svensson, B., and Strand, L., Scand. J. Haematol., 4, 241 (1967).

    Article  CAS  Google Scholar 

  17. Hunt, J. A., and Ingram, V. M., Biochim. Biophys. Acta, 49, 520 (1961).

    Article  CAS  Google Scholar 

  18. Thompson, R. B., Warrington, R. L., and Bell, W. N., Amer. J. Physiol., 268, 198 (1965).

    Google Scholar 

  19. Lehmann, H., Story, P., and Thein, H., Brit. Med. J., 1, 544 (1956).

    Article  CAS  Google Scholar 

  20. White, J. M., Brain, M. C., Lorkin, P. A., Lehmann, H., and Smith, M., Nature, 225, 939 (1970).

    Article  ADS  CAS  Google Scholar 

  21. Huehns, E. R., Hecht, F., Yoshida, A., Stamatoyannopoulos, G., Hartman, J., and Motulsky, A. G., Blood, 36, 209 (1970).

    CAS  PubMed  Google Scholar 

  22. Stamatoyannopoulos, G., Parer, J. T., and Finch, C. A., New England J. Med., 281, 915 (1969).

    Article  Google Scholar 

  23. Miyaji, T., Suzuki, H., Ohba, Y., and Shibata, S., Clin. Chim. Acta, 14, 624 (1966).

    Article  CAS  Google Scholar 

  24. Imai, K., Morimoto, H., Kotani, M., Shibata, S., Miyaji, T., and Matsumoto, K., Biochim. Biophys. Acta, 200, 197 (1970).

    Article  CAS  Google Scholar 

  25. Yanase, T., Hanada, M., Seita, M., Ohta, Y., Imamura, T., Fujimura, T., Kawasaki, K., and Yamaoka, K., Jap. J. Human Genet., 13, 40 (1968).

    CAS  Google Scholar 

  26. Lorkin, P. A., Lehmann, H., Fairbanks, V., Berglund, G., and Leonhardt, T., Biochem. J., 119, 68P (1970).

    Article  CAS  Google Scholar 

  27. Jones, R. T., Osgood, E. E., Brimhall, B., and Koler, R. D., J. Clin. Invest., 46, 1840 (1967).

    Article  CAS  Google Scholar 

  28. Novy, M. J., Edwards, M. J., and Metcalfe, J., J. Clin. Invest., 46, 1848 (1967).

    Article  CAS  Google Scholar 

  29. Reed, C. S., Hampson, R., Gordon, S., Jones, R. T., Novy, M. J., Brimhall, B., Edwards, M. J., and Koler, R. D., Blood, 31, 623 (1968).

    CAS  PubMed  Google Scholar 

  30. Bonaventura, J., and Riggs, A., J. Biol. Chem., 243, 980 (1968).

    CAS  Google Scholar 

  31. Reissman, K. R., Ruth, W. E., and Nomura, T., J. Clin. Invest., 40, 1826 (1961).

    Article  Google Scholar 

  32. Imamura, T., Fujita, S., Ohta, Y., Hanada, M., and Yanase, T., J. Clin. Invest., 48, 2341 (1969).

    Article  CAS  Google Scholar 

  33. Greer, J., J. Mol. Biol., 59, 99 (1971).

    Article  CAS  Google Scholar 

  34. Greer, J., thesis, Univ. Cambridge (1970).

  35. Perutz, M. F., Muirhead, H., Cox, J. M., and Goaman, L. C. G., Nature, 219, 131 (1968).

    Article  ADS  CAS  Google Scholar 

  36. Greer, J., J. Mol. Biol., 59, 107 (1971).

    Article  CAS  Google Scholar 

  37. Bratu, V., Lorkin, P. A., Lehmann, H., and Predescu, C., Biochim. Biophys. Acta (in the press).

  38. King, M. A. R., Wiltshire, B. G., and Lehmann, H., Brit. J. Pharmacol. (in the press).

  39. Yamaoka, K., Blood (in the press).

Download references

Author information

Author notes

  1. H. MORIMOTO

    Present address: Faculty of Engineering Science, Osaka University, Japan

Authors and Affiliations

  1. MRC Abnormal Haemoglobin Unit, Department of Biochemistry, University of Cambridge,

    H. MORIMOTO & H. LEHMANN

  2. MRC Laboratory of Molecular Biology, Cambridge

    M. F. PERUTZ

Authors
  1. H. MORIMOTO
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H. LEHMANN
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. F. PERUTZ
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

MORIMOTO, H., LEHMANN, H. & PERUTZ, M. Molecular Pathology of Human Haemoglobin: Stereochemical Interpretation of Abnormal Oxygen Affinities. Nature 232, 408–413 (1971). https://doi.org/10.1038/232408a0

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1038/232408a0

This article is cited by

Comments

By submitting a comment you agree to abide by our Terms and Community Guidelines. If you find something abusive or that does not comply with our terms or guidelines please flag it as inappropriate.

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing