The realization that a mutation in a haemoglobin gene is at the root of sickle cell anaemia and the discovery of an effective treatment are separated by years. But sickle cell research is a model for how different paths of inquiry can come together with good result.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$209.00 per year
only $17.42 per issue
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
Pauling, L., in Sickle Cell Disease Basic Principles and Clinical Practice (eds. Embury, S.H., Hebbel, R.P., Mohandas, N., Steinberg, M.H.) xvii (Raven Press, New York, 1994).
Pauling, L., Itano, H.A., Singer, S.J. & Wells, I.C. Sickle cell anemia: A molecular disease. Science 110, 543–548 (1949).
Ingram, V.M. A specific chemical difference between the globins of normal human and sickle-cell anemia haemoglobin. Nature 178, 792–794 (1956).
Platt, O.S. et al. Mortality in sickle cell disease: Life expectancy and risk factors for early death. New Engl. J. Med. 330, 1639–1644 (1994).
Wishner, B.C., Ward, K.B., Lattman, E.E. & Love, W.E. Crystal structure of sickle-cell deoxyhemoglobin at 5A resolution. J. mol. Biol. 98, 179–194 (1975).
Orringer, E.P., Abraham, D.J. & Parker, J.C. Development of drug therapy. in Sickle Cell Disease Basic Principles and Clinical Practice (eds. Embury, S.H., Hebbel, R.P., Mohandas, N., Steinberg, M.H.) 861–871 (Raven Press, New York, 1994).
DeSimone, J., Heller, P., Hall, L. & Zwiers, D. 5-Azacytidine stimulates fetal hemoglobin synthesis in anemic baboons. Proc. natn. Acad. Sci. U.S.A. 79, 4428–4431 (1982).
Ley, T.J. et al. 5-Azacytidine selectively increases gamma-globin synthesis in a patient with B+ tha-lassemia. N. Engl. J. Med. 307, 1469–1475 (1982).
Letvin, N.L., Linch, D.C., Beardsley, G.P., Mcln-tyre, K.W. & Nathan, D.G. Augmentation of fetal hemoglobin production in anemic monkeys by hydroxyurea. New Engl. J. Med. 310, 869–873 (1984).
Platt, O.S., Orkin, S.H., Dover, G., Beardsley, G.P., Miller, B. & Nathan, D.G. Hydroxyurea en-hancess fetal hemoglobin production in sickle cell anemia. J. clin. Invest. 74, 652–656 (1987).
Charache, S., Dover, G.J., Moyer, M.A. & Moore, J.W. Hydroxyurea-induced augmentation of fetal hemoglobin production in patients with sickle cell anemia. Blood 69, 109–116 (1987).
Charache, S. et al. Hydroxyurea: Effects on hemoglobin F production in patients with sickle cell anemia. Blood 79, 2555–2565 (1992).
Goldberg, M.A. et al. Treatment of sickle cell anemia with hydroxyurea and erythropoietin. New Engl. J. Med. 323, 366–372 (1990).
Bridges, K. et al. Hydroxyurea changes SS RBC biochemical/biophysical profile to that of SC RBCs. Blood 84 (suppl. 1), 413a (1994).
Castle, W.B. From man to molecule and back to mankind. Semin. Hematol. 13, 159–167 (1976).
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Platt, O. Sickle cell paths converge on hydroxyurea. Nat Med 1, 307–308 (1995). https://doi.org/10.1038/nm0495-307
Issue Date:
DOI: https://doi.org/10.1038/nm0495-307
This article is cited by
-
Hematopoietic cell transplantation for thalassemia and sickle cell disease: past, present and future
Bone Marrow Transplantation (2008)
