growth/size/body
• mice have slower weight gain with a 28% weight reduction by 48 weeks of age compared to controls
• mice weigh 20% less than controls at weaning and 25% less at 20 weeks of age
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weight loss
(
J:76495
)
• mice lose weight after 32 weeks of age
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behavior/neurological
• almost all mice show excessive grooming
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limb grasping
(
J:76495
)
• some mice clasp fore- and hind-limbs when lifted by the tail by 16 weeks of age
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abnormal gait
(
J:76495
)
• mice have a prominent wide gait and a lowered pelvis during grid climbing
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• mice are less active and inquisitive than controls
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• mice have no negative geotaxis after 12 weeks of age
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nervous system
• neuronal degeneration is evident in the cerebellum
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• 20% of Purkinje cells suffer from dendrite atrophy
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• is observed in >50% of pontine nuclei and >50% of dentate nuclei
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• mice have occasional axonal abnormalities and even rarer axonal degeneration
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demyelination
(
J:76495
)
• mice have a minor degree of de- and re- myelination in the sciatic nerves by 6 month of age
• by 14 months of age, extensive myelin abnormalities exist including demyelinated and thinly myelinated axons, and "onion bulb" formations
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Machado-Joseph disease | DOID:1440 |
OMIM:109150 |
J:76495 |