Hepatolenticular Degeneration Model

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Wild Mouse 750
Wild Mouse 750
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Wild Mouse 765
Wild Mouse 765

Hepatolenticular degeneration (Wilson disease) , is an autosomal recessive disorder of copper metabolism caused by pathogenic variants in the ATP7B gene. This gene encodes a P-type ATPase transmembrane copper transporter, with mutations resulting in impaired biliary copper excretion and subsequent toxic copper accumulation in multiple organs, particularly the liver and central nervous system.


● Genetically Engineered Hepatolenticular degeneration Mouse Model

Strain No.
 Strain Name Strain Type Description
T013709 Atp7b-KO Knockout Homozygous Atp7b-KO mice show significant decrease in plasma ceruloplasmin activity and impairment of copper ion tolerance from 6 weeks old. Excess copper accumulation in the liver of 22-week-old Atp7b-KO mice is also detected by LC-MS and Timm staining