Journal article

Heterozygous tx mice have an increased sensitivity to copper loading: Implications for Wilson's disease carriers

Daphne MY Cheah, Yolanda J Deal, Paul FA Wright, Nicole E Buck, Chung Wo Chow, Julian FB Mercer, Katrina J Allen

BIOMETALS | SPRINGER | Published : 2007


Wilson's disease carriers constitute 1% of the human population. It is unknown whether Wilson's disease carriers are at increased susceptibility to copper overload when exposed to chronically high levels of ingested copper. This study investigated the effect of chronic excess copper in drinking water on the heterozygous form of the Wilson's disease mouse model--the toxic milk (tx) mouse. Mice were provided with drinking water containing 300 mg/l copper for 4-7, 8-11, 12-15 or 16-20 months. At the completion of the study liver, spleen, kidney and brain tissue were analyzed by atomic absorption spectroscopy to determine copper concentration. Plasma ceruloplasmin oxidase activity and liver hist..

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