Analysis Tools
mortality/aging
cellular
|
• the first litter is the most severely affected and the severity of the phenotype in pups decreases with each subsequent litter of the dam
|
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)
|
|
• in older homozygotes
|
homeostasis/metabolism
|
• 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
|
pigmentation
|
• pale coat color
|
hematopoietic system
|
• in areas of neuronal necrosis
|
immune system
|
• in areas of neuronal necrosis
|
integument
|
• pale coat color
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Wilson disease | DOID:893 |
OMIM:277900 |
J:130273 | |
