1H NMR-based metabolomics investigation of copper-laden rat: a model of Wilson's disease

Wilson's disease (WD), also known as hepatoleticular degeneration (HLD), is a rare autosomal recessive genetic disorder of copper metabolism, which causes copper to accumulate in body tissues. In this study, rats fed with copper-laden diet are used to render the clinical manifestations of WD, a...

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Bibliographic Details
Main Authors: Jingjing, Xu, Huaizhou, Jiang, Jinquan, Li, Kian, Kai Cheng, Jiyang, Dong, Zhong, Chen
Format: Article
Published: Public Library of Science 2015
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Online Access:http://eprints.utm.my/id/eprint/54925/
http://dx.doi.org/10.1371/journal.pone.0119654
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Summary:Wilson's disease (WD), also known as hepatoleticular degeneration (HLD), is a rare autosomal recessive genetic disorder of copper metabolism, which causes copper to accumulate in body tissues. In this study, rats fed with copper-laden diet are used to render the clinical manifestations of WD, and their copper toxicity-induced organ lesions are studied. To investigate metabolic behaviors of 'decoppering' process, penicillamine (PA) was used for treating copper-laden rats as this chelating agent could eliminate excess copper through the urine. To date, there has been limited metabolomics study on WD, while metabolic impacts of copper accumulation and PA administration have yet to be established.