Wilson's disease is a severe human disorder of copper homoeostasis. The disease is associated with various mutations in the ATP7B gene that encodes a copper-transporting ATPase, and a massive accumulation of copper in the liver and several other tissues. The most frequent disease manifestations include a wide spectrum of liver pathologies as well as neurological and psychiatric abnormalities. A combination of copper chelators and zinc therapy has been used to prevent disease progression; however, accurate and timely diagnosis of the disease remains challenging. Similarly, side effects of treatments are common. To understand better the biochemical and cellular basis of Wilson's disease, several animal models have been developed. This review focuses on genetically engineered Atp7b−/− mice and describes the properties of these knockout animals, insights into the disease progression generated using Atp7b−/− mice, as well as advantages and limitations of Atp7b−/− mice as an experimental model for Wilson's disease.
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December 2008
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Conference Article|
November 19 2008
Atp7b−/− mice as a model for studies of Wilson's disease
Svetlana Lutsenko
Svetlana Lutsenko
1
1Department of Biochemistry and Molecular Biology, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, U.S.A.
1email lutsenko@ohsu.edu
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Publisher: Portland Press Ltd
Received:
July 07 2008
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© The Authors Journal compilation © 2008 Biochemical Society
2008
Biochem Soc Trans (2008) 36 (6): 1233–1238.
Article history
Received:
July 07 2008
Citation
Svetlana Lutsenko; Atp7b−/− mice as a model for studies of Wilson's disease. Biochem Soc Trans 1 December 2008; 36 (6): 1233–1238. doi: https://doi.org/10.1042/BST0361233
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