Sodium-potassium pump, ion fluxes, and cellular dehydration in sickle cell anemia

H. Izumo, S. Lear, M. Williams, R. Rosa, F. H. Epstein

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22 Scopus citations


We studied the role of the sodium-potassium pump in erythrocytes of 12 patients with sickle cell anemia (SS). Ouabain-binding sites per cell and pump-mediated Rb/K uptake were significantly higher in SS patients than in white or black controls. Ouabain-resistant Rb/K influx was also greater than in normal controls or patients with sickle cell trait. Deoxygenation of SS erythrocytes increased ouabain-sensitive Rb/K influx without altering ouabain binding, presumably as the consequence of an increase in the passive influx of sodium. Deoxygenation increased mean corpuscular hemoglobin concentration (MCHC) by 5.5%, and studies of the density distribution of SS cells indicated an increase in highly dense fractions known to contain sickled erythrocytes. Ouabain prevented the rise in MCHC and reduced the percentage of dense cells. These findings indicate a magnified role for the sodium-potassium pump in the pathophysiology of SS erythrocytes and suggest that its inhibition might prove useful in therapy.

Original languageEnglish (US)
Pages (from-to)1621-1628
Number of pages8
JournalJournal of Clinical Investigation
Issue number6
StatePublished - 1987
Externally publishedYes

ASJC Scopus subject areas

  • General Medicine


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