Abstract
We have investigated the levels of fructose 2,6-bisphosphate and its synthesizing enzyme 6-phosphofructo-2-kinase in red blood cells from different congenital anemias. Fructose 2,6-bisphosphate concentration and 6-phosphofructo-2-kinase activity are markedly influenced by the number of reticulocytes in all the cases studied with the exception of homozygous pyruvate kinase deficiency, where no correlation was observed with the percentage of reticulocytes.
MeSH terms
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Anemia, Hemolytic, Congenital / blood*
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Anemia, Hemolytic, Congenital / enzymology
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Anemia, Sickle Cell / blood
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Anemia, Sickle Cell / enzymology
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Erythrocytes / chemistry*
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Erythrocytes / enzymology
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Fructosediphosphates / blood*
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Genotype
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Glucosephosphate Dehydrogenase / blood
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Glucosephosphate Dehydrogenase Deficiency / blood
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Glycolysis
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Hexokinase / blood
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Humans
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Phosphofructokinase-2
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Phosphotransferases / blood
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Pyruvate Kinase / deficiency
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Reticulocytes / chemistry
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Reticulocytes / enzymology
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Spherocytosis, Hereditary / blood
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Spherocytosis, Hereditary / enzymology
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Thalassemia / blood
Substances
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Fructosediphosphates
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fructose 2,6-diphosphate
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Glucosephosphate Dehydrogenase
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Phosphotransferases
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Hexokinase
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Phosphofructokinase-2
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Pyruvate Kinase