Abstract
Sickle cell anemia was first described as a molecular disease by Pauling in 1949 [1] on the basis of the altered electrophoretic mobility of hemoglobin taken from the blood of patients with clinical symptoms. Since then many additional observations have been made on various aspects of the disease [2]. One of the most important is that deoxygenated sickle cell hemoglobin (Hb-S) can aggregate to form well-ordered fibers or microtubules which are seen either within sickled red cells or in gels formed from concentrated, cell-free hemoglobin solutions. The basic geometry of these fibers is known [3, 4], and it seems clear that they are responsible for the distortion, rigidity, and reduced life span of the red cells. However, even though the precise chemical alteration produced by the mutation is known [5], there is still no understanding as to why the replacement of glutamic acid by valine at the sixth position in the β chain causes the aggregation.
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© 1976 D. Reidel Publishing Company, Dordrecht-Holland
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Wodak, S.J., Kahn, P., Dadivanian, A.K., Levinthal, C. (1976). Hemoglobin Interaction in Sickle Cell Fibers: Theoretical Approaches to the Molecular Contacts. In: Pullman, B. (eds) Environmental Effects on Molecular Structure and Properties. The Jerusalem Symposia on Quantum Chemistry and Biochemistry, vol 8. Springer, Dordrecht. https://doi.org/10.1007/978-94-010-1837-1_24
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DOI: https://doi.org/10.1007/978-94-010-1837-1_24
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