Hemolytic anemia in hereditary pyrimidine 5'-nucleotidase deficiency. II. Effect of pyrimidine nucleotides and their derivatives on glycolytic and pentose phosphate shunt enzyme activity

Clinica Chimica Acta; International Journal of Clinical Chemistry
E OdaK R Tanaka


We evaluated the glycolytic intermediate concentrations from the erythrocytes of a patient with hereditary pyrimidine 5'-nucleotidase (P5'N) deficiency. Conclusive evidence for a metabolic block was not found. We evaluated the effects of the pyrimidine (cytidine and uridine) tri- and diphosphate nucleotides (CTP, CDP, UTP, UDP) and the choline and ethanolamine derivatives of CDP (CDP-choline, CDP-ethanolamine) on the activities of key enzymes of the Embden-Meyerhof pathway. CTP and UTP inhibited fructose-6-phosphate competitively for phosphofructokinase and phosphoenolpyruvate competitively for pyruvate kinase. In both cases, the Ki of the pyrimidine nucleotide and Km of the glycolytic substrate were above their intraerythrocytic concentrations. CTP was a competitive inhibitor of ADP for pyruvate kinase with a Ki near its intraerythrocytic concentration. CDP-choline and CDP-ethanolamine had no effect on the activities of Embden-Meyerhof or pentose phosphate shunt enzymes. Thus, the nature of the hemolytic anemia in hereditary P5'N deficiency remains enigmatic.


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Nov 21, 2000·Free Radical Biology & Medicine·H Gurer, N Ercal
Apr 14, 2006·British Journal of Haematology·Alberto ZanellaGiovanna Valentini
Feb 13, 2003·British Journal of Haematology·David C ReesAnthony M Marinaki
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Related Concepts

Anemia, Hemolytic, Congenital
Glucosephosphate Dehydrogenase
Pentose Phosphate Pathway
Phosphogluconate Dehydrogenase

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