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Phenotypes Associated with This Genotype
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp7btx-J mutation (1 available); any Atp7b mutation (34 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• the first litter is the most severely affected and the severity of the phenotype in pups decreases with each subsequent litter of the dam

• mice are moribund by 2 to 3 weeks of age (J:24233)
• however, mice fostered by wild-type dams exhibit increased survival (J:24233)

liver/biliary system
• the mitochondrial in the liver are abnormal at 3 months of age consisting of changes in the width of cristae with some cystic dilation at the tips (J:130273)
• mice exhibit cirrhosis of the liver that is reduced in subsequent generation with continuous fostering to wild-type dams (J:24233)
• in older homozygotes (J:150853)

• copper levels in the brain are increased 1.2-fold at 4 months and 3-fold at 6 months

nervous system
• mice exhibit neuronal necrosis in the dorsal and lateral aspects of the frontal parietal and occipital lobes but not the temporal lobe
• copper levels in the brain are increased 1.2-fold at 4 months and 3-fold at 6 months
• at 1 week of age acute neuronal necrosis, histologically characterized by nuclear pyknosis with cytoplasmic shrinkage and eosinophilia, is found in the dorsal and lateral aspects of the frontal, parietal and occipital lobes, but not the temporal lobe, and the first and second layers of each section of the cortex tend not to be affected while the third to sixth layers are the most affected

• pale coat color (J:150853)

hematopoietic system
• in areas of neuronal necrosis

immune system
• in areas of neuronal necrosis

• pale coat color (J:150853)

Mouse Models of Human Disease
OMIM ID Ref(s)
Wilson Disease 277900 J:130273

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.05
The Jackson Laboratory