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Int. J. Mol. Sci. 2015, 16(3), 6419-6431; doi:10.3390/ijms16036419

Wilson’s Disease: A Comprehensive Review of the Molecular Mechanisms

1,†
,
2,†
,
3
,
4,* and 2,*
1
Department of imaging, the Affiliated Zhongshan Hospital of Dalian University, 6 Jiefang Street, Zhongshan District, Dalian 116001, Liaoning, China
2
Department of Internal Medicine, the Second Hospital of Dalian Medical University, 467 Zhongshan Road, Shahekou District, Dalian 116023, Liaoning, China
3
Department of Biobank, the Sixth People's Hospital of Dalian, 269 Luganghuibai Road, Ganjingzi District, Dalian 116031, Liaoning, China
4
Storr Liver Centre, Westmead Millennium Institute for Medical Research, Faculty of Medicine, the University of Sydney at Westmead Hospital, Westmead, NSW 2145, Australia
These authors contributed equally to this work.
*
Authors to whom correspondence should be addressed.
Academic Editor: Johannes Haybaeck
Received: 7 December 2014 / Revised: 3 March 2015 / Accepted: 3 March 2015 / Published: 20 March 2015
(This article belongs to the Collection Molecular Mechanisms of Human Liver Diseases)
View Full-Text   |   Download PDF [1489 KB, uploaded 20 March 2015]   |  

Abstract

Wilson’s disease (WD), also known as hepatolenticular degeneration, is an autosomal recessive inherited disorder resulting from abnormal copper metabolism. Reduced copper excretion causes an excessive deposition of the copper in many organs such as the liver, central nervous system (CNS), cornea, kidney, joints, and cardiac muscle where the physiological functions of the affected organs are impaired. The underlying molecular mechanisms for WD have been extensively studied. It is now believed that a defect in P-type adenosine triphosphatase (ATP7B), the gene encoding the copper transporting P-type ATPase, is responsible for hepatic copper accumulation. Deposited copper in the liver produces toxic effects via modulating several molecular pathways. WD can be a lethal disease if left untreated. A better understanding of the molecular mechanisms causing the aberrant copper deposition and organ damage is the key to developing effective management approaches. View Full-Text
Keywords: Wilson’s disease; ATP7B gene; copper metabolism; molecular mechanism Wilson’s disease; ATP7B gene; copper metabolism; molecular mechanism
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Wu, F.; Wang, J.; Pu, C.; Qiao, L.; Jiang, C. Wilson’s Disease: A Comprehensive Review of the Molecular Mechanisms. Int. J. Mol. Sci. 2015, 16, 6419-6431.

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