hemoglobinopathies/glutathione

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Puslapis 1 nuo 19 rezultatus

Influence of glutathione S-transferase A1, P1, M1, T1 polymorphisms on oral busulfan pharmacokinetics in children with congenital hemoglobinopathies undergoing hematopoietic stem cell transplantation.

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BACKGROUND Busulfan (BU), often used in high dose for myeloablation before hematopoietic stem cell transplantation (HSCT), has been implicated in certain HSCT toxicities, including the occurrence of hepatic veno-occlusive disease (HVOD). In addition to weight and age, gene polymorphisms in specific

Erythrocyte reduced glutathione level and its stability in hemoglobinopathies and iron deficiency anemia.

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Partial glutathione reductase deficiency as a cause of diverse clinical manifestations in a family with unstable hemoglobin (Hemoglobin Haná, β63(E7) His-Asn).

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Hemoglobin Haná [β63(E7) His-Asn] is an unstable hemoglobin variant that was described in a Czech proband and her sister with Heinz body hemolytic anemia. The mother bearing the same mutation was asymptomatic; nevertheless, all three carriers had the same proportion of the mutant globin chains.

Glutathione S-transferase T1-null seems to be associated with graft failure in hematopoietic SCT.

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Hematopoietic SCT (HSCT) from HLA-matched donors is sometimes complicated by GVHD or graft rejection, because of mismatched mHA. This study presents data suggesting the involvement of glutathione S-transferase theta-1 (GSTT1), a phase II detoxifying enzyme encoded by GSTT1, in Ab-mediated rejection

Relationship between oxidative stress, glutathione S-transferase polymorphisms and hydroxyurea treatment in sickle cell anemia.

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This study evaluated the oxidative stress and antioxidant capacity markers in sickle cell anemia (SCA) patients with and without treatment with hydroxyurea. We assessed GSTT1, GSTM1 and GSTP1 polymorphisms in patients and a control group. The study groups were composed of 48 subjects without

Pharmacologic interception of heme: a potential therapeutic strategy for the treatment of β thalassemia?

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Pro-oxidant effects of hemoglobin-derived heme and iron contribute to the progressive damage observed in β thalassemic and sickle (HbS) red blood cells. Agents that prevent heme/iron release and inhibit their redox activity might diminish such injury. Consequently, the inhibitory effects of

N-acetylcysteine amide (AD4) attenuates oxidative stress in beta-thalassemia blood cells.

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Many aspects of the pathology in beta-hemoglobinopathies (beta-thalassemia and sickle cell anemia) are mediated by oxidative stress. In the present study we tested a novel thiol compound, N-acetylcysteine amide (AD4), the amide form of N-acetyl cysteine (NAC) for its antioxidant effects. Using

The Homozygous Hemoglobin EE Variant Is Associated with Poorer Riboflavin Status in Cambodian Women of Reproductive Age

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Background: Riboflavin is required for erythropoiesis, which is increased in people with hemoglobinopathies due to increased hemolysis and erythrocyte turnover. Dietary intake and status of riboflavin is poor in Cambodia, where

The role of hemoglobin heme loss in Heinz body formation: studies with a partially heme-deficient hemoglobin and with genetically unstable hemoglobins.

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A number of mutant hemoglobins are inordinately unstable, denaturing in circulating red cells into Heinz bodies, resulting in congenital Heinz body hemolytic anemia (CHBHA). We have emphasized that most such hemoglobins involve amino acid substitutions at sites neighboring the heme group of the

Assessment of the red cell proteome of young patients with unexplained hemolytic anemia by two-dimensional differential in-gel electrophoresis (DIGE).

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Erythrocyte cytosolic protein expression profiles of children with unexplained hemolytic anemia were compared with profiles of close relatives and controls by two-dimensional differential in-gel electrophoresis (2D-DIGE). The severity of anemia in the patients varied from compensated (i.e., no

Fermented papaya preparation as redox regulator in blood cells of beta-thalassemic mice and patients.

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Many aspects of the pathology in beta-hemoglobinopathies (beta-thalassemia and sickle cell anemia) are mediated by oxidative stress. Fermented papaya preparation (FPP) was tested for its antioxidant effects: the scavenging effect was determined spectrofluorometrically in a cell-free system using

Amelioration of oxidative stress in red blood cells from patients with beta-thalassemia major and intermedia and E-beta-thalassemia following administration of a fermented papaya preparation.

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In beta-hemoglobinopathies, such as beta-thalassemia (thal) and sickle cell anemia, the primary defects are mutations in the beta-globin gene. However, many aspects of the pathophysiology are mediated by oxidative stress. Fermented papaya preparation (FPP), a natural health food product obtained by

Erythrocyte oxidative stress markers in children with sickle cell disease.

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OBJECTIVE To determine eight parameters of oxidative stress markers in erythrocytes from children with sickle cell disease and compare with the same parameters in erythrocytes from healthy children, since oxidative stress plays an important role in the pathophysiology of sickle cell disease and

Oxidative stress and labile plasmatic iron in anemic patients following blood therapy.

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OBJECTIVE To determine the plasmatic iron content and evaluate the oxidative stress (OS) markers in subjects receiving blood therapy. METHODS Thirty-nine individuals with unspecified anemia receiving blood transfusions and 15 healthy subjects were included in the study. Anemic subjects were divided

Relationship between oxidative stress, ferritin and insulin resistance in sickle cell disease.

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BACKGROUND Sickle cell disease (SCD) is a hereditary hemoglobinopathy characterized by hemolytic anemia. The oxidative phenomena play a significant role in its pathophysiology. Blood transfusions are a therapeutic mainstay in SCD and repeated transfusions can result in iron overload. There is little

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