Favism: a hemolytic disease associated with increased superoxide dismutase and decreased glutathione peroxidase activities in red blood cells.
Red blood cells of favism patients with acute hemolytic crisis have markedly more superoxide dismutase (superoxide:superoxide oxidoreductase, EC 1.15.1.1) and less glutathione peroxidase (glutathione:hydrogenperoxide oxidoreductase, EC 1.11.1.9) than either normal controls, glucose-6-phosphate dehydrogenase-deficient subjects or favism patients outside hemolytic crisis. This altered value of the two enzyme activities is not due to increased reticulocyte content of blood. The electrophoretic triplet pattern of superoxide dismutase is also changed, with significant increase of the most positively charged band. Similar modifications of the two enzyme activities are observed after treatment of normal red blood cells with high concentrations of divicine and ascorbate, which are redox compounds that are contained in fava seeds. This treatment produces no hemolysis, but leads to hemolysis if the treated cells are resuspended in the homologous plasma. These results suggest a possible role of active oxygen species in the development of favism.
D001205:Ascorbic Acid / Q000494:pharmacology; D004591:Electrophoresis, Polyacrylamide Gel; D004905:Erythrocyte Aging; D004912:Erythrocytes / Q000201:enzymology*; D005236:Favism / Q000097:blood / Q000201:enzymology*; D005979:Glutathione Peroxidase / Q000097:blood*; D006461:Hemolysis; D006801:Humans; D010084:Oxidation-Reduction; D011744:Pyrimidinones / Q000494:pharmacology; D012156:Reticulocytes / Q000201:enzymology; D013482:Superoxide Dismutase / Q000097:blood*
D011744:Pyrimidinones; C026511:divicine; D005979:Glutathione Peroxidase; D013482:Superoxide Dismutase; D001205:Ascorbic Acid