RED CELLS Molecular basis of glutathione reductase deficiency in human blood cells
نویسندگان
چکیده
1Sanquin Research and Landsteiner Laboratory, Academic Medical Centre, University of Amsterdam, The Netherlands; 2Department of Pediatrics, Erasmus Medical Centre, Location Sophia, Rotterdam, The Netherlands; 3Pediatric Department, Medical Center Rijnmond-Zuid, Location Clara, Rotterdam, The Netherlands; 4Emma Childrens Hospital, Academic Medical Centre, University of Amsterdam, The Netherlands; 5Biochemie-Zentrum Heidelberg, Heidelberg University, Germany
منابع مشابه
Molecular basis of glutathione reductase deficiency in human blood cells.
Hereditary glutathione reductase (GR) deficiency was found in only 2 cases when testing more than 15 000 blood samples. We have investigated the blood cells of 2 patients (1a and 1b) in a previously described family suffering from favism and cataract and of a novel patient (2) presenting with severe neonatal jaundice. Red blood cells and leukocytes of the patients in family 1 did not contain an...
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By ERNEST BEUTLER AND MARY K. Y. Yu m T HE EXACT ROLE of reduced glutathione ( OSH ) in the economy of the red blood cell remains to be defined. Drug-induced hemolytic anemia due to glucose-6-phosphate dehydrogenase deficiency is associated with lowt and with unstable2 0S1-I. Yet, a condition has been described in which red cell glutathione is virtually absent with only mild hemolysis being obs...
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Abstract Background and Objective: Glutathione as a master antioxidant plays a key role in eliminating free radicals from your body and in preserving cell membrane integrity esp. blood cells. We aimed to assess the Erythrocyte Glutathione level of favism and non-favism neonates. Material and Methods: This study was conducted in Ekbatan hospitals of Hamadan (2009) on 15 favism patients and 60...
متن کاملFamilial deficiency of glutathione reductase in human blood cells.
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 ...
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The mechanism by which glutathione reductase (GR) activity is regulated in relation to fiavin metabolism was studied in red cells of normal adults, cord blood, and patients with severe metabolic disorders using spectrophotometnc, fluorometnc, and radiochemical methods. The increased activity of GR in red cells of adult patients with severe hepatic cirrhosis, chronic uremia, and glucose-6-phosph...
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