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pubmed:abstractText |
The cellular concentration of Hb S plays a central role in the kinetic of Hb S polymerization and cell sickling. Blood of patients with homozygous sickle cell (SS) anemia contains a variable fraction of cells which are markedly dehydrated and have increased Hb S concentration. Since a decrease in cellular Hb S concentration reduces Hb S polymerization and sickling, the study of the processes leading to sickle cell dehydration has important pathophysiological and therapeutic implications. Sickle cell dehydration is due to cellular loss of K and Cl. K loss in sickle cells can take place via either the Ca(2+)-activated K+ channel, or the K-Cl cotransport, or the combined effect of oxidative damage and deformation of the red cell membrane. Inhibitors of K transport through these pathways could be used to prevent dehydration of sickle cells in vivo, provided that they can be administered safely.
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