Leukocyte function and characterization of leukocyte glucose-6-phosphate dehydrogenase in Sicilian mutants

Pediatric Research
G SchiliròS Marino


Nine Sicilian children known to be deficient in glucose-6-phosphate dehyrdrogenase (G6PD) were studied to see if there were anomalies of bactericidal activity in peripheral blood phagocytes. The type of deficiency was established. The G6PD levels in the leukocyte were found to be 26% of the controls (0.094 +/- 0.03, normal controls 0.360 +/- 0.12). The Michaelis constant for NADP and glucose-6-phosphate (G6P) was lower than the control. Conversely, the utilization of the analogous 2-deoxyglucose-6-phosphate (2dG6P) and galactose-6-phosphate (Ga16P) was higher. The thermostability of the enzyme in the deficient subjects was lower and the pH optima (8 and 9.5) were different from the controls. An identical electrophoretic pattern was found in both normal and deficient subjects. The bactericidal activity in the deficient subjects was normal. There was no difference in the results of nitroblue tetrazolium (NBT) tests in either group.


Jul 28, 2016·The Journal of Allergy and Clinical Immunology·Ulrich SilerJanine Reichenbach
Sep 1, 1980·Acta paediatrica Scandinavica·G SchiliròG Pizzarelli

Related Concepts

Blood Bactericidal Activity
Glucosephosphate Dehydrogenase
Deficiency of glucose-6-phosphate Dehydrogenase
Hydrogen-Ion Concentration

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