Severe dysfunction of respiratory chain and cholesterol metabolism in Atp7b(-/-) mice as a model for Wilson disease.

@article{Sauer2011SevereDO,
  title={Severe dysfunction of respiratory chain and cholesterol metabolism in Atp7b(-/-) mice as a model for Wilson disease.},
  author={Sven Wolfgang Sauer and Uta Merle and Silvana Opp and Dorothea Haas and Georg Friedrich Hoffmann and Wolfgang Stremmel and Juergen Guenther Okun},
  journal={Biochimica et biophysica acta},
  year={2011},
  volume={1812 12},
  pages={1607-15}
}
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 changes in Atp7b(-/-) mice focusing on liver and brain. Mutant mice showed strongly elevated copper and iron levels. Age-dependently decreasing hepatic reduced glutathione levels along with increasing… CONTINUE READING
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