Plasma Copper and Antioxidant Status in Wilson's Disease

Ogihara, Hiromi; Ogihara, Tohru; Miki, Masayuki; Yasuda, Hiroshi; Mino, Makoto

doi:10.1203/00006450-199502000-00016

Cited by 116 publications

(52 citation statements)

References 30 publications

(46 reference statements)

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“…The chief mechanism is thought to be caused by hemolysis (17). Moreover, patients with vitamin E deficiency have been reported to be more susceptible to hemolysis associated with oxidative stress (18).…”

Section: Discussionmentioning

confidence: 99%

Randomized Controlled Open-Label Trial of Vitamin E-Bonded Polysulfone Dialyzer and Erythropoiesis-Stimulating Agent Response

Sanaka¹,

Mochizuki²,

Kinugasa³

et al. 2013

Clinical Journal of the American Society of Nephrology

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the VEESA Study Group Summary Background and objectives A 1-year multicenter prospective randomized controlled study was conducted on the effects of vitamin E-bonded polysulfone dialyzers on erythropoiesis-stimulating agent response in hemodialysis patients.Design, setting, participants, & measurements Major inclusion criteria were use of high-flux polysulfone dialyzers with 50-70 ml/min b 2 -microglobulin clearance over 3 months, transferrin saturation over 20%, same erythropoiesisstimulating agent for over 3 months, and hemoglobin at 10-12 g/dl. Hemodialysis patients were placed in four interventional groups: two hemoglobin ranges (10.0-10.9 or 11.0-11.9 g/dl) and two dialyzers. Patients were randomly assigned by central registration to a vitamin E-bonded polysulfone dialyzers or polysulfone control group. Primary end point was relative erythropoiesis resistance index at baseline between groups at 12 months. Erythropoiesis resistance index was defined as total weekly erythropoiesis-stimulating agent dose divided by hemoglobin.Results There were no statistically significant differences in age or sex. There was no significant difference in relative erythropoiesis resistance index between vitamin E-bonded polysulfone dialyzers and control groups at 12 months (vitamin E-bonded polysulfone dialyzers: 1.1, control: 1.3). The vitamin E-bonded polysulfone dialyzers group showed better relative erythropoiesis resistance index than the control group at 11.0-11.9 g/dl hemoglobin (vitamin E-bonded polysulfone dialyzers: 1.0, control: 1.4 at 12 months, significant difference) but no difference at 10.0-10.9 g/dl hemoglobin. ConclusionsThe overall relative erythropoiesis resistance index showed no difference between the vitamin Ebonded polysulfone dialyzers and control groups, although the change in relative erythropoiesis resistance index differed according to hemoglobin level.

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Section: Discussionmentioning

confidence: 99%

Randomized Controlled Open-Label Trial of Vitamin E-Bonded Polysulfone Dialyzer and Erythropoiesis-Stimulating Agent Response

Sanaka¹,

Mochizuki²,

Kinugasa³

et al. 2013

Clinical Journal of the American Society of Nephrology

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“…Several experiments using HepG2 cells have shown that proteins involved in anti-oxidant defenses in this cell line are altered dramatically by chronic copper exposure (Strand et al, 1998;Jiménez et al, 2002). However, in humans, the magnitude of copper-derived oxidative stress and the state of anti-oxidant defense system in et Summer and Eisenburg, 1985;Ogihara et al, 1995;von Herbay et al, 1994). Recently, oxidative stress and anti-oxidant defense systems in various liver diseases have been studied extensively, especially those in nonalcoholic fatty liver.…”

Section: Introductionmentioning

confidence: 99%

Children's toxicology from bench to bed - Liver Injury (3): Oxidative stress and anti-oxidant systems in liver of patients with wilson disease

Nagasaka¹,

Takayanagi²,

Tsukahara

2009

J. Toxicol. Sci.

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-The role of oxidative stress in the pathogenesis of liver disease in Wilson disease (WD), a genetic disorder characterized by excess hepatic deposition of copper, which generates free radicals, remains unclear. This study investigates oxidative stress on the liver and hepatic anti-oxidant responses in WD using liver specimens from affected patients showing mild liver damage (group I, n = 3), moderate or greater liver damage (group II, n = 5) and fulminant hepatic failure (group III, n = 5), and from asymptomatic carriers (n = 2). Decreased ratios of reduced glutathione (GSH) to oxidized glutathione (GSSG) and increased thiobarbituric acid reactive substance (TBARS), a lipid peroxidation product, were found in every affected patient, especially in groups II and III patients. Activities and protein expressions of Mn-dependent superoxide dismutase (Mn-SOD), CuZn-SOD, and catalase were decreased in all patients, especially in group III patients. Glutathione peroxidase activity was decreased only in group III patients. Asymptomatic carriers without any clinical manifestations showed normal TBARS level and GSH/GSSG ratio with increases in both GSH and GSSG levels. Their CuZn-SOD, Mn-SOD and catalase activities were increased. These results suggest that excessive copper-derived oxidants contribute to development and progression of liver disease in WD.

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“…In addition, oxidative damage is recognized as an important cause of hepatic injury in a variety of liver toxicoses (1,4,17,23,27,35). The following study was therefore undertaken to assess whether this mechanism of cellular damage could be involved in the pathogenesis of prolonged, sublethal MCLR exposure.…”

Section: Introductionmentioning

confidence: 99%

Hepatic Oxidative Stress Following Prolonged Sublethal Microcystin LR Exposure

1999

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Microcystin LR (MCLR) is a naturally occurring protein phosphatase inhibitor and potent hepatotoxin produced by strains of Microcystis aeruginosa. Although its acute toxicity has been well characterized, little is known about its chronic effects. In this study, we sought to acquire evidence that oxidative stress may play a role in the pathogenesis of prolonged sublethal MCLR toxicity. Twelve rats (3 per group) weighing on average 185 g were exposed to 1 of 3 different concentrations of MCLR (16, 32, and 48 μg/kg/day) or to saline via intraperitoneal osmotic pumps for 28 days. Histologic evidence of dose-dependent hepatic inflammation was seen, including infiltration of centrilobular regions by lymphocytes, macrophages, and neutrophils, centrilobular fibrosis, apoptosis, and steatosis. Analysis of lipid peroxidation products revealed a dose-dependent increase in malondialdehyde concentrations with an approximate 4-fold increase in the livers of the high-dose rats over those of the saline-treated controls. Livers from MCLR-exposed rats were more sensitive than those of controls to the cytotoxic effects of the organic oxidizing agent tert-butyl hydroperoxide, based on an MTT (3-[dimethylthiazol-2-yl]-2,5-diphenyl-tetrazolium bromide) viability assay. These histopathologic and biochemical findings indicate that oxidative stress may play a significant role in the pathogenesis of chronic MCLR toxicosis.

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Plasma Copper and Antioxidant Status in Wilson's Disease

Cited by 116 publications

References 30 publications

Randomized Controlled Open-Label Trial of Vitamin E-Bonded Polysulfone Dialyzer and Erythropoiesis-Stimulating Agent Response

Randomized Controlled Open-Label Trial of Vitamin E-Bonded Polysulfone Dialyzer and Erythropoiesis-Stimulating Agent Response

Children's toxicology from bench to bed - Liver Injury (3): Oxidative stress and anti-oxidant systems in liver of patients with wilson disease

Hepatic Oxidative Stress Following Prolonged Sublethal Microcystin LR Exposure

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