PMID- 3034977 OWN - NLM STAT- MEDLINE DCOM- 19870710 LR - 20191210 IS - 0021-9738 (Print) IS - 0021-9738 (Linking) VI - 79 IP - 6 DP - 1987 Jun TI - Sodium-potassium pump, ion fluxes, and cellular dehydration in sickle cell anemia. PG - 1621-8 AB - 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. FAU - Izumo, H AU - Izumo H FAU - Lear, S AU - Lear S FAU - Williams, M AU - Williams M FAU - Rosa, R AU - Rosa R FAU - Epstein, F H AU - Epstein FH LA - eng GR - AM-18078/AM/NIADDK NIH HHS/United States GR - HL-15157/HL/NHLBI NIH HHS/United States GR - RR-01032/RR/NCRR NIH HHS/United States PT - Journal Article PT - Research Support, U.S. Gov't, P.H.S. PL - United States TA - J Clin Invest JT - The Journal of clinical investigation JID - 7802877 RN - 0 (Receptors, Drug) RN - 0 (cardiac glycoside receptors) RN - 5ACL011P69 (Ouabain) RN - 9NEZ333N27 (Sodium) RN - EC 7.2.2.13 (Sodium-Potassium-Exchanging ATPase) RN - MLT4718TJW (Rubidium) RN - RWP5GA015D (Potassium) RN - S88TT14065 (Oxygen) SB - IM MH - Adolescent MH - Adult MH - Anemia, Sickle Cell/*metabolism MH - Body Water/metabolism MH - Erythrocyte Indices MH - Erythrocytes/*metabolism MH - Humans MH - Middle Aged MH - Ouabain/metabolism/pharmacology MH - Oxygen/blood MH - Potassium/*metabolism MH - Receptors, Drug/analysis MH - Rubidium/metabolism MH - Sodium/*metabolism MH - Sodium-Potassium-Exchanging ATPase/antagonists & inhibitors/*metabolism PMC - PMC424484 EDAT- 1987/06/01 00:00 MHDA- 1987/06/01 00:01 PMCR- 1987/06/01 CRDT- 1987/06/01 00:00 PHST- 1987/06/01 00:00 [pubmed] PHST- 1987/06/01 00:01 [medline] PHST- 1987/06/01 00:00 [entrez] PHST- 1987/06/01 00:00 [pmc-release] AID - 10.1172/JCI112998 [doi] PST - ppublish SO - J Clin Invest. 1987 Jun;79(6):1621-8. doi: 10.1172/JCI112998.