Carbamyl Phosphate Modification of Hemoglobin S Structure Resulting in Altered Sickling

  • Lorraine M. Kraus
  • Asri Rasad
  • Alfred P. Kraus
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 28)


Interest has been elicited in the mechanism involved in the successful reversal and prevention of the sickling of erythrocytes containing Hb SS. Urea, reported by Nalbandian (1971) and by McCurdy (1971), cyanate, reported by Cerami and Manning (1971), and carbamyl phosphate (carbamyl P), reported by Kraus and Kraus (1971) have been successfully used to inhibit the sickling phenomenon. Urea is proposed to act reversibly by interfering with the hydrophobic bonding of hemoglobin molecules, (Murayama, 1971), while cyanate is reported to act irreversibly through the carbamyl ation of the amino terminal valines, (Cerami and Manning, 1971).


Sickle Cell Sickle Cell Anemia Patient Cyanic Acid Finger Print Hemoglobin Molecule 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Allen, C. M., and Jones, M. E. (1964). Decomposition of Carbamyl- phosphate in Aqueous Solutions. Biochemistry 3: 1238.PubMedCrossRefGoogle Scholar
  2. Anson, D. W., and Mirsky, A. E. (1930). Protein coagulation and its reversal: the preparation of insoluble globin, soluble globin and heme. J. Gen. Physiol. 13: 469.PubMedCrossRefGoogle Scholar
  3. Boyer, S. H., Fainer, D. C., and Naughton, M. A. (1963). Myoglobin: Inherited structural variation in man. Science 140: 1228.PubMedCrossRefGoogle Scholar
  4. Bunn, H. F., May, M. H., Kocholaty, W. F. and Shields, C. E. (1969). Hemoglobin function in stored blood. J. Clin. Invest. 48: 311.PubMedCrossRefGoogle Scholar
  5. Cerami, A., and Manning, J. M. (1971). Potassium Cyanate as an Inhibitor of the Sickling of Erythrocytes In Vitro. Proc. Nat. Acad. Sci. U.S.A. 68: 1180.CrossRefGoogle Scholar
  6. Clegg, J. B., Naughton, M. A., and Weatherall, D. J. (1965). An improved method for the characterization of human haemoglobin mutants: identification of α2β295Glu, haemoglobin N ( Baltimore ). Nature 207: 945.PubMedCrossRefGoogle Scholar
  7. Drabkin, D. L. (1946). Spectrophotometry studies. XIV. The crystallographic and optical properties of the hemoglobin of man in comparison with those of other species. J. Biol. Chem. 164: 703.PubMedGoogle Scholar
  8. Gillette, P. N., Manning, J. M., and Cerami, A. (1971). Increased Survival of Sickle-Cell Erythrocytes after Treatment In Vitro with Sodium Cyanate. Proc. Nat. Acad. Sci. U.S.A. 68: 2791.CrossRefGoogle Scholar
  9. Ingram, V. M. (1958). Abnormal human Haemoglobins. I. The comparison of normal human and sickle cell haemoglobins by “finger-printing”. Biochim. Biophys. Acta. 28: 539.PubMedCrossRefGoogle Scholar
  10. Kraus, A. P., Langston, M. F. Jr., and Lynch, B. L. (1968). Red Cell Phosphoglycerate Kinase Deficiency. Biochem. Biophys. Res. Comm. 30: 173.PubMedCrossRefGoogle Scholar
  11. Kraus, L. M., Miyaji, T., Iuchi, I., and Kraus, A. P. (1966). Characterization of α23GluNH2 in Hemoglobin Memphis. Hemoglobin Memphis/S, a New Variant of Molecular Disease. Biochemistry 5: 3701.PubMedCrossRefGoogle Scholar
  12. Kraus, L. M. and Kraus, A. P. (1971). Carbamyl Phosphate Mediated Inhibition of the Sickling of Erythrocytes In Vitro. Biochem. Biophys. Res. Comm. 44: 1381.PubMedGoogle Scholar
  13. McCurdy, P. R., and Mahmood, L. (1971). Urea Therapy for Sickle Cell Disease. Clin. Res. 19: 75.Google Scholar
  14. Murayama, M. (1971). In “Molecular Aspects of Sickle Cell Hemoglobin” (R. M. Nalbandian, ed.). Chas. C. Thomas Co., Springfield, 111.Google Scholar
  15. Nalbandian, R. M., Ed. (1971). Molecular Aspects of Sickle Cell Hemoglobin. Charles C. Thomas Co., Springfield, 111.Google Scholar
  16. Smithies, O. (1959). An improved procedure for starch gel electrophoresis: further variations in the serum proteins of normal individuals. Biochem. J. 71: 585.PubMedGoogle Scholar

Copyright information

© Plenum Press, New York 1972

Authors and Affiliations

  • Lorraine M. Kraus
    • 1
    • 2
  • Asri Rasad
    • 1
    • 2
  • Alfred P. Kraus
    • 1
    • 2
  1. 1.Department of BiochemistrySickle Cell Center, University of Tennessee Medical UnitsMemphisUSA
  2. 2.Department of Medicine Hematology SectionSickle Cell Center, University of Tennessee Medical UnitsMemphisUSA

Personalised recommendations