glucose-6-phosphate dehydrogenase (g6pd) deficiency is the most prevalent enzymopathy in mankind. it has sex-linked in­heritance. this enzyme exists in all cells.  g6pd deficiency increases the sensitivity of red blood cells to oxidative dam­age. g6pd deficiency was discovered in 1950 when some people suffered hemolytic anemia as a result of taking antimalar­ial drugs (primaquin). most people w...

The susceptibility to hemolysis following administration of primaquine, naphthalene and other drugs, as well as after the ingestion of fava beans was described in individuals with a defect in their erythrocyte metabolism (1, 2). These erythrocytes manifest a number of abnormalities, namely, a low reduced glutathione (GSH) level, a glutathione instability when exposed to acetylphenyl hydrazine, ...

Cytochemical staining remains an efficient way of identifying females who are heterozygous for the X chromosome-linked glucose-6-phosphate dehydrogenase (G6PD) gene. G6PD is highly polymorphic with certain alleles resulting in low intracellular G6PD activity in red blood cells. Low intracellular G6PD activity is associated with a risk of severe hemolysis when exposed to an oxidative stress such...

Glucose-6-phosphate dehydrogenase (G6PDH; EC 1.1.1.49) deficiency is one of the most common human enzymopathies throughout the world. Although most affected individuals are asymptomatic, there is a risk of neonatal jaundice and acute hemolytic anemia which can be triggered by infection, some pharmaceuticals and, in older individuals, eating fava beans. We characterized the molecular basis of G6...

A virtually complete absence of glutathione reductase activity was found in the erythrocytes of all three children (one male, two females) from a consanguineous marriage. Intermediate values were found in the erythrocytes of both parents. The enzyme activity could not be restored either by addition of FAD in vitro or by administration of riboflavin in vivo. The amount of reduced glutathione in ...

BACKGROUND AND OBJECTIVES Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common erythrocytic enzymatic disorder in Italy and is characterized by wide clinical, biochemical and molecular variability. We studied the clinical and hematologic data from 54 G6PD-deficient, unrelated males from the Apulia region. DESIGN AND METHODS Analyses for enzymatic activity, G6PD electrophores...

A famous painting entitled The Fruit Seller (Fruittivendola) painted in 1580 by Vincenzo Campi located in the Pinacoteca di Brera, Milan, depicts an elegant lady vender with a lapful of peaches holding a bunch of black grapes presiding over more than a dozen different fruits and vegetables for sale (Fig. 1), including several cucurbits. A large yellow, oblate, ribbed pumpkin (Cucurbita pepo L. ...

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary enzyme defect in humans, caused by a mutation X-linked gene encoding G6PD. The G6PD plays an important role to produced reducing agents which maintain reduced glutathione through pentose phosphate pathway. On ingestion of Faba Bean (Vicia faba L.), vicine and convicine are hydrolyzed β -glucosidase divicine isoura...

The biochemical events that take place during acute hemolysis of G6PD-deficient subjects in favism are far from being elucidated. Evidence is here reported for a constantly and heavily disordered calcium homeostasis in the erythrocytes from seven favic patients. The abnormality, ie, a significantly impaired calcium ATPase activity and a parallel marked increase of intracellular calcium levels, ...