Consequences of copper accumulation in the livers of the Atp7b-/- (Wilson disease gene) knockout mice.

@article{Huster2006ConsequencesOC,
  title={Consequences of copper accumulation in the livers of the Atp7b-/- (Wilson disease gene) knockout mice.},
  author={Dominik Huster and Milton J. Finegold and Clinton T Morgan and Jason L Burkhead and Randal R Nixon and Scott M. Vanderwerf and Conrad T. Gilliam and Svetlana Lutsenko},
  journal={The American journal of pathology},
  year={2006},
  volume={168 2},
  pages={423-34}
}
Wilson disease is a severe genetic disorder associated with intracellular copper overload. The affected gene, ATP7B, has been identified, but the molecular events leading to Wilson disease remain poorly understood. Here, we demonstrate that genetically engineered Atp7b-/- mice represent a valuable model for dissecting the disease mechanisms. These mice, like Wilson disease patients, have intracellular copper accumulation, low-serum oxidase activity, and increased copper excretion in urine… CONTINUE READING

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