Glucose-6-phosphate dehydrogenase (G-6-PD) Hamburg, a new variant with chronic nonspherocytic haemolytic anaemia |
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Authors: | Manfred Gahr,Werner Schr ter |
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Affiliation: | Manfred Gahr,Werner Schröter |
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Abstract: | Abstract. A new variant of G-6-PD with chronic non-spherocytic haemolytic anaemia and very low activity, named G-6-PD Hamburg, was partially purified and biochemically characterized. It was found to have very high lability, an unusually high Km for G-6-P (2000 μM), increased utilization rates for 2-desoxy G-6-P (133%) and galactose-6-phosphate (87%) and an abnormal pH-activity curve. The electrophoretic mobility seemed to be normal. The leukocytes also revealed diminished G-6-PD activity. No impairment of bactericidal activity of neutrophilic granulocytes, as shown by a normal nitroblue tetrazolium reduction, could be demonstrated. |
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Keywords: | Glucose-6-phosphate dehydrogenase deficiency chronic nonspherocytic haemolytic anaemia nitroblue tetrazolium reduction test phagocytosis |
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