nervous system
• histology of the brain found dead neurons in the cerebral cortex, hippocampus, and hypothalamus
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• cross sections of the inner ear shows enlarged spiral ganglion but normal organ of Corti
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• histology of the brain finds large and small dystrophic axons in many places
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behavior/neurological
mortality/aging
• all homozygotes are dead by 4 weeks of age
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• most homozygotes die by 3 weeks of age, but some survive to 4 weeks of age
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• intercrosses of heterozygotes produce fewer than 17% homozygous pups
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growth/size/body
• by three weeks of age homozygotes become thinner overall with a sunken stomach
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hearing/vestibular/ear
• auditory brainstem response analysis at two and three weeks of age found no response, indicative of complete deafness
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digestive/alimentary system
• wavy colonic crypts, edematous villi in the small intesting and bubbly cytoplasm of transitional epithelium were found in a female assessed at 3.5 weeks of age
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• hyperkeratosis of the stomach was found in a female assessed at 3.5 weeks of age
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integument
• hair follicles of a female assessed at 3.5 weeks of age show all to be in catagen
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endocrine/exocrine glands
immune system
hematopoietic system
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
neuronal ceroid lipofuscinosis 10 | DOID:0110725 |
OMIM:610127 |
J:229377 |