Adenosine Triphosphate Metabolism in Hereditary Spherocytosis*

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Abstract
The adenosine triphosphate (ATP) content of incubated whole blood or washed red cells declines more rapidly in samples obtained from patients with hereditary spherocytosis (HS) than in normal samples, indicating a greater rate of ATP utilization by HS red cells. The increased utilization of ATP by HS red cells is partly caused by increased activity of the Na-K pump because ouabain, which blocks active cation transport, has a greater effect on glucose and ATP utilization in HS red cells than in normal cells. When active cation transport in normal and HS cells is completely blocked by ouabain, there remains an increased rate of ATP and glucose consumption by HS cells compared to normal, suggesting that the HS erythrocyte has an increased energy requirement in addition to active cation transport. The corrective effect of glucose in diminishing autohemolysis of HS blood is abolished by ouabain, indicating that the utilization of ATP for active cation transport is of paramount importance in protecting the HS erythrocyte against hemolysis.