ATP Metabolism in Pyruvate Kinase Deficient Erythrocytes

Elevated levels of ATP were observed in pyruvate kinase deficient red cells from 5 patients despite increased ATP requirements because of increased ATPase activity. Increased red cell ATPase activity was associated with an excess of intracellular sodium. Residual aerobic glycolysis in reticulocytes did not contribute significantly toward ATP generation in these patients’ red cells. Their hemolytic anemia cannot be ascribed to a lack of high energy phosphate. Conversely, hemolysis in vitro appears to require a high energy phosphate dependent metabolic process.