Enolase Activity of Erythrocytes in Hereditary Spherocytosis

Abstract

Tabechian, Altman and Young¹ have shown that red blood cells from patients with hereditary spherocytosis are more sensitive to fluoride than normal red blood cells with respect to the ³²P-orthophosphate exchange reaction between the extra- and intra-cellular compartments. From these results they concluded that the enzymes enolase or adenosine triphosphatase are the most likely to be defective in the red blood cells of patients with hereditary spherocytosis. Prankerd² investigated the adenosine triphosphatase activity of such cells and found this enzyme to be normally active. The possibility that the defect involves enolase is supported by some unpublished results of Altman and Young. However, glycolytic intermediates have been shown to be normal^3,4. These results strongly indicate that an enzymatic defect in the Embden–Meyerhof pathway is unlikely.