The Experts below are selected from a list of 360 Experts worldwide ranked by ideXlab platform
David J. Jollow - One of the best experts on this subject based on the ideXlab platform.
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Favism: Effect of Divicine on Rat Erythrocyte Sulfhydryl Status, Hexose Monophosphate Shunt Activity, Morphology, and Membrane Skeletal Proteins
2013Co-Authors: David C. Mcmillan, Laura J. C. Bolchoz, David J. JollowAbstract:Favism is an acute anemic crisis that can occur in susceptible individuals who ingest fava beans. The fava bean pyrimidine aglycone divicine has been identified as a hemotoxic constituent; however, its mechanism of toxicity remains unknown. We have shown recently that divicine can induce a favic-like response in rats and that divicine is directly toxic to rat red cells. In the present study, we have examined the effect of hemotoxic concentrations of divicine on rat erythrocyte sulfhydryl status, hexose monophosphate (HMP) shunt activity, morphology, and membrane skeletal proteins. In vitro exposure of rat red cells to divicine markedly stimulated HMP shunt activity and resulted in depletion of reduced glutathione with concomitant formation of glutathioneprotein mixed-disulfides. Examination of divicine-treated red cells by scanning electron microscopy revealed transformation of the cells to an extreme echinocytic morphology. SDS-PAGE and immunoblottin
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Favism effect of divicine on rat erythrocyte sulfhydryl status hexose monophosphate shunt activity morphology and membrane skeletal proteins
Toxicological Sciences, 2001Co-Authors: David C. Mcmillan, Laura J. C. Bolchoz, David J. JollowAbstract:Favism is an acute anemic crisis that can occur in susceptible individuals who ingest fava beans. The fava bean pyrimidine aglycone divicine has been identified as a hemotoxic constituent; however, its mechanism of toxicity remains unknown. We have shown recently that divicine can induce a favic-like response in rats and that divicine is directly toxic to rat red cells. In the present study, we have examined the effect of hemotoxic concentrations of divicine on rat erythrocyte sulfhydryl status, hexose monophosphate (HMP) shunt activity, morphology, and membrane skeletal proteins. In vitro exposure of rat red cells to divicine markedly stimulated HMP shunt activity and resulted in depletion of reduced glutathione with concomitant formation of glutathione-protein mixed-disulfides. Examination of divicine-treated red cells by scanning electron microscopy revealed transformation of the cells to an extreme echinocytic morphology. SDS-PAGE and immunoblotting analysis of the membrane skeletal proteins indicated that hemotoxicity was associated with the apparent loss of skeletal protein bands 2.1, 3, and 4.2, and the appearance of membrane-bound hemoglobin. Treatment of divicine-damaged red cells with dithiothreitol reversed the protein changes, which indicated that the observed alterations were due primarily to the formation of disulfide-linked hemoglobin-skeletal protein adducts. The data suggest that oxidative modification of hemoglobin and membrane skeletal proteins by divicine may be key events in the mechanism underlying Favism.
Paolo Arese - One of the best experts on this subject based on the ideXlab platform.
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no red blood cell damage and no hemolysis in g6pd deficient subjects after ingestion of low vicine convicine vicia faba seeds
Blood, 2018Co-Authors: Valentina Gallo, Oleksii A Skorokhod, Luigi Felice Simula, Tiziana Marrocco, Elisa Tambini, Evelin Schwarzer, Pascal Marget, Gerard Duc, Paolo AreseAbstract:TO THE EDITOR: Favism, or “favic crisis,” is a potentially life-threatening acute hemolysis elicited in carriers of low-activity glucose 6-phosphate dehydrogenase (G6PD) variants by ingestion of raw faba bean ( Vicia faba L) (FB) seeds.[1][1],[2][2] In 2 surveys of hemolytic crises because of
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Favism and Glucose-6-Phosphate Dehydrogenase Deficiency
The New England journal of medicine, 2018Co-Authors: Lucio Luzzatto, Paolo AreseAbstract:Favism and Glucose-6-Phosphate Dehydrogenase Deficiency When persons with G6PD deficiency eat fava beans, acute hemolytic anemia may develop. It is caused by the generation of free radicals from the metabolism of glucosides in the beans. The free radicals damage red cells, resulting in intravascular and extravascular lysis.
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life and death of glucose 6 phosphate dehydrogenase g6pd deficient erythrocytes role of redox stress and band 3 modifications
Transfusion Medicine and Hemotherapy, 2012Co-Authors: Paolo Arese, Valentina Gallo, Antonella Pantaleo, Franco TurriniAbstract:G6PD catalyzes the first, pace-making reaction of pentosephosphate cycle (PPC) which produces NADPH. NADPH maintains glutathione and thiol groups of proteins and enzymes in the reduced state which is essential for protection against oxidative stress. Individuals affected by G6PD deficiency are unable to regenerate reduced glutathione (GSH) and are undefended against oxidative stress. G6PD deficiency accelerates normal senescence and enhances the precocious removal of chronologically young, yet biologically old cells. The term hemolytic anemia is misleading because RBCs do not lyse but are removed by phagocytosis. Acute hemolysis by fava bean ingestion in G6PD deficient individuals (Favism) is described being the best-studied natural model of oxidant damage. It bears strong analogies to hemolysis by oxidant drugs or chemicals. Membrane alterations observed in vivo during Favism are superimposable to changes in senescent RBCs. In summary, RBC membranes isolated from favic patients contained elevated amounts of complexes between IgG and the complement fragment C3b/C3c and were prone to vesiculation. Anti-band 3 IgG reacted to aggregated band 3-complement complexes. In Favism extensive clustering of band 3 and membrane deposition of hemichromes were also observed. Severely damaged RBCs isolated from early crises had extensive membrane cross-bonding and very low GSH levels and were phagocytosed 10-fold more intensely compared to normal RBCs.
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life and death of glucose 6 phosphate dehydrogenase g6pd deficient erythrocytes role of redox stress and band 3 modifications
Transfusion Medicine and Hemotherapy, 2012Co-Authors: Paolo Arese, Valentina Gallo, Antonella Pantaleo, Franco TurriniAbstract:Summary G6PD catalyzes the first, pace-making reaction of pentosephosphate cycle (PPC) which produces NADPH. NADPH maintains glutathione and thiol groups of proteins and enzymes in the reduced state which is essential for protection against oxidative stress. Individuals affected by G6PD deficiency are unable to regenerate reduced glutathione (GSH) and are undefended against oxidative stress. G6PD deficiency accelerates normal senescence and enhances the precocious removal of chronologically young, yet biologically old cells. The term hemolytic anemia is misleading because RBCs do not lyse but are removed by phagocytosis. Acute hemolysis by fava bean ingestion in G6PD deficient individuals (Favism) is described being the beststudied natural model of oxidant damage. It bears strong analogies to hemolysis by oxidant drugs or chemicals. Membrane alterations observed in vivo during Favism are superimposable to changes in senescent RBCs. In summary, RBC membranes isolated from favic patients contained elevated amounts of complexes between IgG and the complement fragment C3b/C3c and were prone to vesiculation. Anti-band 3 IgG reacted to aggregated band 3-complement complexes. In Favism extensive clustering of band 3 and membrane deposition of hemichromes were also observed. Severely damaged RBCs isolated from early crises had extensive membrane cross-bonding and very low GSH levels and were phagocytosed 10-fold more intensely compared to normal RBCs. Schlusselworter G6PD · G6PD-Defizienz · Band 3 · Hamolyse · Favismus
David C. Mcmillan - One of the best experts on this subject based on the ideXlab platform.
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Favism: Effect of Divicine on Rat Erythrocyte Sulfhydryl Status, Hexose Monophosphate Shunt Activity, Morphology, and Membrane Skeletal Proteins
2013Co-Authors: David C. Mcmillan, Laura J. C. Bolchoz, David J. JollowAbstract:Favism is an acute anemic crisis that can occur in susceptible individuals who ingest fava beans. The fava bean pyrimidine aglycone divicine has been identified as a hemotoxic constituent; however, its mechanism of toxicity remains unknown. We have shown recently that divicine can induce a favic-like response in rats and that divicine is directly toxic to rat red cells. In the present study, we have examined the effect of hemotoxic concentrations of divicine on rat erythrocyte sulfhydryl status, hexose monophosphate (HMP) shunt activity, morphology, and membrane skeletal proteins. In vitro exposure of rat red cells to divicine markedly stimulated HMP shunt activity and resulted in depletion of reduced glutathione with concomitant formation of glutathioneprotein mixed-disulfides. Examination of divicine-treated red cells by scanning electron microscopy revealed transformation of the cells to an extreme echinocytic morphology. SDS-PAGE and immunoblottin
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Favism effect of divicine on rat erythrocyte sulfhydryl status hexose monophosphate shunt activity morphology and membrane skeletal proteins
Toxicological Sciences, 2001Co-Authors: David C. Mcmillan, Laura J. C. Bolchoz, David J. JollowAbstract:Favism is an acute anemic crisis that can occur in susceptible individuals who ingest fava beans. The fava bean pyrimidine aglycone divicine has been identified as a hemotoxic constituent; however, its mechanism of toxicity remains unknown. We have shown recently that divicine can induce a favic-like response in rats and that divicine is directly toxic to rat red cells. In the present study, we have examined the effect of hemotoxic concentrations of divicine on rat erythrocyte sulfhydryl status, hexose monophosphate (HMP) shunt activity, morphology, and membrane skeletal proteins. In vitro exposure of rat red cells to divicine markedly stimulated HMP shunt activity and resulted in depletion of reduced glutathione with concomitant formation of glutathione-protein mixed-disulfides. Examination of divicine-treated red cells by scanning electron microscopy revealed transformation of the cells to an extreme echinocytic morphology. SDS-PAGE and immunoblotting analysis of the membrane skeletal proteins indicated that hemotoxicity was associated with the apparent loss of skeletal protein bands 2.1, 3, and 4.2, and the appearance of membrane-bound hemoglobin. Treatment of divicine-damaged red cells with dithiothreitol reversed the protein changes, which indicated that the observed alterations were due primarily to the formation of disulfide-linked hemoglobin-skeletal protein adducts. The data suggest that oxidative modification of hemoglobin and membrane skeletal proteins by divicine may be key events in the mechanism underlying Favism.
Laura J. C. Bolchoz - One of the best experts on this subject based on the ideXlab platform.
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Favism: Effect of Divicine on Rat Erythrocyte Sulfhydryl Status, Hexose Monophosphate Shunt Activity, Morphology, and Membrane Skeletal Proteins
2013Co-Authors: David C. Mcmillan, Laura J. C. Bolchoz, David J. JollowAbstract:Favism is an acute anemic crisis that can occur in susceptible individuals who ingest fava beans. The fava bean pyrimidine aglycone divicine has been identified as a hemotoxic constituent; however, its mechanism of toxicity remains unknown. We have shown recently that divicine can induce a favic-like response in rats and that divicine is directly toxic to rat red cells. In the present study, we have examined the effect of hemotoxic concentrations of divicine on rat erythrocyte sulfhydryl status, hexose monophosphate (HMP) shunt activity, morphology, and membrane skeletal proteins. In vitro exposure of rat red cells to divicine markedly stimulated HMP shunt activity and resulted in depletion of reduced glutathione with concomitant formation of glutathioneprotein mixed-disulfides. Examination of divicine-treated red cells by scanning electron microscopy revealed transformation of the cells to an extreme echinocytic morphology. SDS-PAGE and immunoblottin
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Favism effect of divicine on rat erythrocyte sulfhydryl status hexose monophosphate shunt activity morphology and membrane skeletal proteins
Toxicological Sciences, 2001Co-Authors: David C. Mcmillan, Laura J. C. Bolchoz, David J. JollowAbstract:Favism is an acute anemic crisis that can occur in susceptible individuals who ingest fava beans. The fava bean pyrimidine aglycone divicine has been identified as a hemotoxic constituent; however, its mechanism of toxicity remains unknown. We have shown recently that divicine can induce a favic-like response in rats and that divicine is directly toxic to rat red cells. In the present study, we have examined the effect of hemotoxic concentrations of divicine on rat erythrocyte sulfhydryl status, hexose monophosphate (HMP) shunt activity, morphology, and membrane skeletal proteins. In vitro exposure of rat red cells to divicine markedly stimulated HMP shunt activity and resulted in depletion of reduced glutathione with concomitant formation of glutathione-protein mixed-disulfides. Examination of divicine-treated red cells by scanning electron microscopy revealed transformation of the cells to an extreme echinocytic morphology. SDS-PAGE and immunoblotting analysis of the membrane skeletal proteins indicated that hemotoxicity was associated with the apparent loss of skeletal protein bands 2.1, 3, and 4.2, and the appearance of membrane-bound hemoglobin. Treatment of divicine-damaged red cells with dithiothreitol reversed the protein changes, which indicated that the observed alterations were due primarily to the formation of disulfide-linked hemoglobin-skeletal protein adducts. The data suggest that oxidative modification of hemoglobin and membrane skeletal proteins by divicine may be key events in the mechanism underlying Favism.
Scott N Reading - One of the best experts on this subject based on the ideXlab platform.
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Favism the commonest form of severe hemolytic anemia in palestinian children varies in severity with three different variants of g6pd deficiency within the same community
Blood Cells Molecules and Diseases, 2016Co-Authors: Scott N Reading, Mahmoud M Sirdah, Mohammad E Shubair, Benjamin Nelson, Mustafa S Alkahlout, Jamal M Altayeb, Lina N Aboud, Maysaa Abu ShabanAbstract:Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common genetic abnormality known to predispose to acute hemolytic anemia (AHA), which can be triggered by certain drugs or infection. However, the commonest trigger is fava beans (Vicia faba) ingestion, causing AHA (Favism), which may be life-threatening especially in children. G6PD deficiency is genetically highly heterogeneous, as nearly 200 different mutations have been observed. We have investigated the hematological features of acute Favism in the Palestinian Gaza community that is characterized by the polymorphic coexistence of three different G6PD deficiency genes (G6PD A-, G6PD Cairo, G6PD Med). We have found by comparison to the general population (485 adults and 466 newborns) that children with Favism, in terms of relative frequency, G6PD A- was under-represented, whereas G6PD Med was over-represented. We also found that the severity of anemia was significantly greater with G6PD Med and G6PD Cairo than with G6PD A-; and with G6PD Cairo, compared to the other two variants, there was greater hyperbilirubinemia, as well as persistence of mild anemia and reticulocytosis for as long as 4months after recovery from Favism. This is the first report determining a differential impact of different G6PD mutations on the clinical features of Favism in the same population and the same environment.
