Severe dysfunction of respiratory chain and cholesterol metabolism in Atp7b−/− mice as a model for Wilson disease
Wilson disease (WD) is caused by mutations of the WD gene ATP7B resulting in copper accumulation in different tissues. WD patients display hepatic and neurological disease with yet poorly understood pathomechanisms. Therefore, we studied age-dependent (3, 6, 47weeks) biochemical and bioenergetical c...
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| Main Authors: | , , , , , , |
|---|---|
| Format: | Article (Journal) |
| Language: | English |
| Published: |
2 September 2011
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| In: |
Biochimica et biophysica acta. Molecular basis of disease
Year: 2011, Volume: 1812, Issue: 12, Pages: 1607-1615 |
| ISSN: | 1879-260X |
| DOI: | 10.1016/j.bbadis.2011.08.011 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.bbadis.2011.08.011 Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S0925443911001931 |
| Author Notes: | Sven W. Sauer, Uta Merle, Silvana Opp, Dorothea Haas, Georg F. Hoffmann, Wolfgang Stremmel, Jürgen G. Okun |
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Metallothionein is elevated in liver and duodenum of Atp7b(−/−) mice
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