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Science 8 July 1966:
Vol. 153. no. 3732, pp. 145 - 149
DOI: 10.1126/science.153.3732.145
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Articles

Molecular Mechanism of Red Cell "Sickling"

Makio Murayama 1

1 Laboratory of Physical Biology, National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda, Maryland.

Precision scale models of sickle-cell hemoglobin molecules indicate that the genetic substitution of valine for glutamic acid at the 6th position in the two beta chains allows an intramolecular hydrophobic bond to form. This changes the conformation in such a way as to allow molecular stacking. Optical rotatory dispersion studies and the restilts of suLbjection of Hb S solution to temperature change and to propane are consistent with the presence of such a bond. Examination of sickled erythrocytes in a magnetic field and in polarized light indicates that the Hb S molecules are aligned iiz sitil. Filaments interpreted as hollow cables of six Hb S monofilaments have been demonstrated by electron microscopy.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
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Cold Spring Harb Symp Quant Biol 61, 495-509
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Rational approaches to chemotherapy: antisickling agents.
I. Klotz, D. Haney, and L. King (1981)
Science 213, 724-731
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Sickle hemoglobin aggregation: a new class of inhibitors.
Votano JR, M Gorecki, and A Rich (1977)
Science 196, 1216-1219
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Ultrastructural Features of Erythrocyte and Hemoglobin Sickling.
J. G. White (1974)
Arch Intern Med 133, 545-562
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The Genetics of Sickle Cell Anemia and Related Syndromes.
D. L. Rucknagel (1974)
Arch Intern Med 133, 595-606
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Ultrastructure of the Normal and Hemoglobinopathic Red Blood Cell Membrane: Freeze-Etching and Stereoscan Electron Microscopic Studies.
L. S. Lessin, W. N. Jensen, and P. Klug (1972)
Arch Intern Med 129, 306-319
   Abstract »    PDF »
Hemoglobin Interaction: Modification of Solid Phase Composition in the Sickling Phenomenon.
J. F. Bertles, R. Rabinowitz, and J. Dobler (1970)
Science 169, 375-377
   Abstract »    PDF »
Cytochrome P-420: Tubular Aggregates from Hepatic Microsomes.
D. W. Shoeman, J. G. White, and G. J. Mannering (1969)
Science 165, 1371-1372
   Abstract »    PDF »
Microincision of Sickled Erythrocytes by a Laser Beam.
W. N. Jensen, P. A. Bromberg, and M. C. Bessis (1967)
Science 155, 704-707
   Abstract »    PDF »


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