Co‐ordinated Increase of Sodium Leak and Sodium Pump in Hereditary Spherocytosis

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Abstract
Summary: The presence of an increased sodium leak into hereditary spherocytes up to three times normal has been confirmed from measurement of 22Na+ influx into red cells. 22Na+ influx was not identical to the net inward leak of Na+ but the two kept a constant relationship since influx was about double the net Na+ gain in both normal and abnormal cells incubated with ouabain. The intracellular concentration of Na+ ions in fresh cells from hereditary spherocytes was identical to that in normal red cells. Hereditary spherocytes showed increased maximal activity of a membrane ATP‐ase inhibited by ouabain and the Na+ concentration giving half‐maximal activation of this ATP‐ase was the same for spherocytes and normal cells. When the 22Na+ influx into red cells from nine patients with hereditary spherocytosis was compared with maximal activity of cation pump ATP‐ase in the same cells a significant correlation was obtained (r= 0.78, P > 0.01). The results indicate some linkage between the increase in the Na+ leak and functional number of active cation pumps in the membrane of hereditary spherocytes.