Abnormal phosphoenolpyruvate transport in erythrocytes of hereditary spherocytosis.

The rate of phosphoenolpyruvate transport in erythrocytes from patients with hereditary spherocytosis and from healthy individuals was examined in a 0.1 M citrate buffer (pH 6.1 at 37 degrees C) containing 10 mM phosphoenolpyruvate and 10 mM NaF. The rate in erythrocytes from patients with hereditary spherocytosis was 0.09 +/- 0.02 mumol/min/ml of cells (mean +/- SD, n = 8), whereas the rate in erythrocytes from healthy individuals was 0.23 +/k- 0.03 mumol/min/ml of cells (mean +/- SD, n = 8). The lower rate of transport in erythrocytes of hereditary spherocytosis could not be described to a different glycolytic response to NaF or to a difference in cell age distribution. This phenomenon seemed to be a reflection of some abnormality in the erythrocyte membrane from patients with hereditary spherocytosis.