Regulation of glucose-6-phosphate dehydrogenase activity in red blood cells from hemolytic and nonhemolytic variant subjects.
نویسندگان
چکیده
منابع مشابه
MOLECULAR IDENTIFICATION OF THE MOST PREVALENT MUTATION OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD) GENE IN DEFICIENT PATIENTS IN GILAN PROVINCE
Glucose-6-Phosphate Dehydrogenase (G6PD) is a cytosolic enzyme which its main function is to produce NADPH in the red blood cells by controlling the step from Glucose-6-Phosphate to 6-Phospho gluconate in the pentose phosphate pathway. G6PD deficiency is the most common X-chromosome linked hereditary enzymopathy in the world, that result in reduced enzyme activity and more than 125 different mu...
متن کاملG6PD San Francisco: a new variant of glucose-6-phosphate dehydrogenase associated with congenital nonspherocytic hemolytic anemia.
Congenital nonspherocytic hemolytic anemia in an adult male of Scandinavian ancestry was associated with virtual absence of G6PD activity in red cells. Characterization of G6PD purified from leukocytes using standard WHO techniques revealed diminished electrophoretic mobility, marked lability on heating at 46 degrees C, normal pH optimum and utilization of alternate substrates (2-deoxy G6P, D-a...
متن کاملHemolysis Induced by Glucose-6-Phosphate Dehydrogenase Deficiency and Its Association with Sex in Children
Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme disorder in human. The aim of this study was to determine the prevalence of G6PD deficiency among children and evaluate its association with ABO/Rh blood groups. Method: Blood samples of 3401 asymptomatic children were analyzed and compared with 317 children who were admitted to hospital because of hemolys...
متن کاملDiagnosis of erythrocyte glucose-6-phosphate dehydrogenase deficiency in the negro male despite hemolytic crisis.
E RYTHROCYTE GLUCOSE-6-PHOSPHATE DEHYDROGENASE ( G-6PD ) DEFICIENCY is a frequent clinically-significant genetically-determined abnormality of man. On the basis of gene frequency of the enzyme deficiency, about 3,000,000 Negroes in the United States carry genes for this disorder.1 When exposed to certain drugs and chemical agents, clinically normal persons with G-6-PD deficiency experience hemo...
متن کاملG-6-PD Long Prairie: a new glucose-6-phosphate dehydrogenase mutant exhibiting normal sensitivity to inhibition by NADPH and accompanied by nonspherocytic hemolytic anemia.
The enzymatic properties of a new glucose-6-phosphate dehydrogenase (G-6-PD) variant (G-6-PD Long Prairie) were studied in a white patient with chronic nonspherocytic hemolysis. The red cells were found to have 2.3%-7.7% normal enzymatic activity. The mutant enzyme exhibited marked heat instability, an increased pH optimum, a moderately decreased Km for G-6-P, and increased utilization of 2-deo...
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ورودعنوان ژورنال:
- Blood
دوره 41 6 شماره
صفحات -
تاریخ انتشار 1973