Glucose-6-Phosphate Dehydrogenase
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چکیده
Description Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is the most common human enzyme deficiency that mainly affects the red blood cells (RBCs). A defect in G6PD enzyme leads to the destruction of premature RBCs causing hemolytic anemia because the body can not compensate the destroyed RBCs. Thus, the affected individuals show jaundice (paleness, yellowing of the skin and whites of the eyes), dark urine, fatigue, shortness of breath, and a rapid heart rate. However, many patients are asymptomatic. Hemolytic anemia associated with G6PD deficiency is most often triggered by bacterial or viral infections and by oxidative drugs. Also, eating fava beans or inhaling pollen from fava plants increase hemolysis, therefore, G6PD deficiency is sometimes called favism. All these factors will increase the reactive oxygen species level leading to faster hemolysis. Individuals with G6PD deficiency are at risk to have serious conditions that may lead to death, if they are not properly treated. Identification of the causative mutations and the adequate therapeutic strategy are established on the basis of molecular analysis.
منابع مشابه
Inhibition of Human Erythrocyte Glucose 6-Phosphate Dehydrogenase by Cadmium, Nickel and Aluminium
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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...
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Glucose 6-phosphate dehydrogenase (G6PD) was purified from Streptomyces aureofaciens and inactivated with butanedione and diethylpyrocarbonate. Incubation of the enzyme with butanedione resulted in a rapid activity loss (80%) within 5 min, followed by a slow phase using a molar ratio to enzyme concentration of 100. Fluorescence studies showed a conformational change in the butanedione-modified ...
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Glucose 6-phosphate dehydrogenase (G6PD) from Streptomyces aureofaciens was purified and denatured in 6 M urea. Denaturation led to complete dissociation of the enzyme into its inactive monomers, 98% loss of the enzyme activity, about 30% decrease in the protein fluorescence and a 10 nm red shift in the emission maximum. Dilution of urea-denatured enzyme resulted in regaining of the enzyme acti...
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In order to explore the nature of glucose-6-phosphate dehydrogenase (G6PD) deficiency in one of the coastal provinces of the Caspian Sea (Mazandaran) in Iran, we have analysed the G6PD gene in 74 unrelated G6PD-deficient males (2-6 year children) with a history of Favism, by using PCR and subsequent digestion by appropriate restriction enzymes, looking for the presence of certain known mutation...
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