Glycerol-3-phosphate dehydrogenase activity in the red cells of patients
with thalassemia
P Fessas, NP Anagnou and D Loukopoulos
L-alpha-Glycerol-3-phosphate dehydrogenase (EC 1.1.1.8) has been reported
to be absent in the erythrocytes of normal adults, but can be found in
those of cord blood and of thalassemia major. The aid of this study was to
investigate whether there is any relation between GDH and gamma-chain
synthesis. Erythrocyte GDH activity was determined on 118 different blood
samples. It was undetectable in normal adult erythrocytes and definitely
high in cord blood cells (23.6 UI/10(11) RBC). Considerable GDH activity
was also noted in patients with thalassemia major (11.0 IU10(11) RBC) as
well as in cases with pronounced reticulocytosis (11.4 IU/10(11) RBC). Red
cells from beta- thalassemia heterozygotes exhibited moderate but distinct
GDH activity (5.2 IU/10(11) RBC). After fractionation into young and old
erythrocyte populations, clearly higher GDH activity was found in the
younger cells; however, there was no significant correlation with the
reticulocyte count. Presence of reticulocytes alone appears insufficient to
explain the values obtained in cord blood and the thalassemias, especially
heterozygous. Furthermore, no direct correlation between GDH and fetal
hemoglobin (HbF) was obtained in cord and thalassemic erythrocytes.
Volume 55,
Issue 4,
pp. 564-569,
04/01/1980
Copyright © 1980 by The American Society of Hematology
