Abstract
IN paroxysmal nocturnal haemoglobinuria (PNH) a membrane abnormality renders the red blood cell (RBC) extremely sensitive to the lytic action of complement1. The nature of the abnormality is unknown: an alteration of the cell lipids has been postulated on the basis of the results of some lipid determinations2–8 and the greater tendency of lipids to form peroxides after exposure to ultraviolet light or H2O29–11. Alteration of the membrane proteins has also been proposed as the cause of the cell abnormality12,13. It has been found that treatment with some sulphydryl compounds renders normal RBCs similar to those in PNH in many respects14. In particular, they display a sensitivity to complement lysis twenty-fold greater than normal15 and have a very low acetylcholinesterase activity16. Among the sulphydryl compounds so far examined14,17–20 the radio-protector AET (2-amino-ethyl-isothiouronium bromide) has been shown to be the most effective in producing the PNH-like abnormality21. The PNH-like alteration induced in normal cells by sulphydryl compounds has not yet been identified, although it seems to involve the breaking of membrane disulphide bonds21. We have studied PNH and AET RBC membranes in parallel using sodium dodecyl sulphate (SDS) electrophoresis in Polyacrylamide gels. Our experiments show further similarities between PNH and AET cells, and indicate that, in both, the alteration (or at least part of it) is located in the protein moiety of the membrane, where an abnormal polypeptide chain is demonstrable.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
We are sorry, but there is no personal subscription option available for your country.
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
References
Rosse, W. F., and Dacie, J. V., J. clin. Invest., 45, 736 (1966).
De Gier, J., van Deenen, L. L. M., Verloop, M. C., and van Gaste, C., Br. J. Haemat., 10, 246 (1964).
Formijne, P., Poulie, N. J., and Rodbard, J. A., Clin. Chim. Acta, 2, 25 (1957).
Harris, I. M., Prankerd, T. A. J., and Westerman, M. P., Br. med. J., 2, 1276 (1957).
Leibetseder, F., and Ahrens, E. H., jun., Br. J. Haemat., 5, 356 (1959).
Lovelock, J. E., and Prankerd, T. A. J., Proc. seventh int. Congr. Hemat., Rome 1958, 2, 434 (II Pensiero Scientifico, Rome, 1960).
Meriwether, W. D., and Mengel, C. E., Nature, 210, 91 (1966).
Munn, J. I., and Crosby, W. H., Proc. Soc. exp. Biol., 96, 480 (1957).
Mengel, C. E., and Kann, H. E., jun., Clin. Res., 13, 278 (1965).
Mengel, C. E., Kann, H. E., jun., and Meriwether, W. D., J. clin. Invest., 46, 1715 (1967).
Meriwether, W. D., and Mengel, C. E., Nature, 210, 91 (1966).
Crosby, W. H., Blood, 8, 444 (1953).
Thomas, S. A., Shapiro, B., and Green, J. W., Fed. Proc., 31, 341 (1972).
Sirchia, G., Ferrone, S., and Mercuriali, F., Blood, 25, 502 (1965).
Sirchia, G., and Dacie, J. V., Nature, 215, 747 (1967).
Sirchia, G., Ferrone, S., Milani, R., and Mercuriali, F., Blood, 28, 98 (1966).
Kann, H. E., jun., Mengel, C. E., Meriwether, W. D., and Ebbert, L., Blood, 32, 49 (1968).
Sirchia, G., Mercuriali, F., and Ferrone, S., Experientia, 24, 495 (1968).
De Sandre, G., Cortési, C., Corrocher, R., Falezza, G., and Perona, G., Br. J. Haemat., 18, 551 (1970).
Sandre, G., Vettore, L., Corrocher, R., Cortesi, S., and Perona, G., Br. J. Haemat., 15, 437 (1968).
Sirchia, G., Zanella, A., Perrella, M., Mercuriali, F., and Ferrone, S., Experientia, 28, 191 (1972).
Metz, J., Bradlow, B. A., Lewis, S. M., and Dacie, J. V., Br. J. Haemat., 6, 372 (1960).
Myhre, E., and Flatmark, T., Br. J. Haemat., 8, 48 (1962).
Kan, S. Y., and Gardner, F. H., Blood, 25, 759 (1965).
Dodge, J. T., Mitchell, C., and Hanahan, D. J., Archs Biochem. Biophys., 100, 119 (1963).
Laemli, U. K., Nature, 227, 680 (1970).
Dameshek, W., Bull. New Engl. med. Cen., 4, 224 (1942).
Haut, A., and Taylor, E. H., Blood, 28, 994 (1966).
Fairbanks, G., Steck, T. L., and Wallach, D. F. H., Biochemistry, 10, 2606 (1971).
Rosenberg, S. A., and Guidotti, G., J. biol. Chem., 244, 5118 (1969).
Rosenberg, S. A., and Guidotti, G., in Red Cell Membrane, Structure and Function (edit, by Jamieson, G. A., and Green-wait, T. J.), 93 (Lippincott, Philadelphia, 1969).
Lenard, J., Biochemistry, 9, 1129 (1970).
Lenard, J., Biochemistry, 9, 5037 (1970).
Bender, W. W., Garan, H., and Berg, H. C., J. molec. Biol., 58, 783 (1971).
Bretscher, M. S., J. molec. Biol., 59, 351 (1971).
Glossmann, H., and Neville, D. M., jun., J. biol. Chem., 246, 6339 (1971).
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
RIGHETTI, P., PERRELLA, M., ZANELLA, A. et al. The Membrane Abnormality of the Red Cell in Paroxysmal Nocturnal Haemoglobinuria. Nature New Biology 245, 273–276 (1973). https://doi.org/10.1038/newbio245273a0
Received:
Revised:
Issue Date:
DOI: https://doi.org/10.1038/newbio245273a0
This article is cited by
-
Abnormality of erythrocyte membrane protein in a case of congenital stomatocytosis
Klinische Wochenschrift (1977)