Children's toxicology from bench to bed - Liver Injury (3): Oxidative stress and anti-oxidant systems in liver of patients with wilson disease
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- Nagasaka Hironori
- Division of Metabolism, Chiba Children’s Hospital
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- Takayanagi Masaki
- Division of Metabolism, Chiba Children’s Hospital
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- Tsukahara Hirokazu
- Department of Pediatrics, Fukui Medical University
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説明
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.
収録刊行物
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- The Journal of Toxicological Sciences
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The Journal of Toxicological Sciences 34 (Special Issue 2), SP229-SP236, 2009
一般社団法人 日本毒性学会
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詳細情報 詳細情報について
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- CRID
- 1390001204902309376
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- NII論文ID
- 110007337716
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- NII書誌ID
- AN00002808
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- ISSN
- 18803989
- 03881350
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- NDL書誌ID
- 10370128
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- 本文言語コード
- en
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- データソース種別
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- JaLC
- NDL
- Crossref
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- 使用不可