behavior/neurological
• in the hind limb clasp test, 6-month old mice splay their hind limbs inwardly and grasp solid objects and 12-month old mice have about 2 times higher hind limb clasping
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• 12-month old mice exhibit a higher number of foot slippages than 6-month old mice on a beam walk test
(J:302080)
• the mean latency to fall in the ledge test is shorter in both 6- and 12-month old mice compared to wild-type mice, with 12-month old mice showing greater difficulty walking along the ledge and slipping and falling about 2 times more than wild-type mice
(J:302080)
• in the narrow beam-walking test, mice exhibit an increase in the number of slips as early as 2-6 months of age and after 12 months of age, mice show progressive difficulty in traversing the beam, with a delay in completing the task
(J:302256)
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• mice show slight dragging of the hind limbs at several months of age which progressively worsens with age
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cellular
• cortical neurons grown on poly-D-lysine-coated plates show only marginal changes in morphology during the first 3 days and show reduced rate of formation of ganglial networks at 96 hours, indicating reduced growth of cortical neurons
• cortical neurons fail to respond to EGF-induced growth and maturation
• however, proliferation rate of cortical neurons in response to EGF is similar to wild-type neurons
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nervous system
• cortical neurons grown on poly-D-lysine-coated plates show a reduction in the number of neurons with axonal branches
(J:302080)
• cortical neurons form fewer branches per axon
(J:302080)
• cerebral cortical neurons grown on glass cover slips show increased axonal branching
(J:302256)
• however, the number of primary dendrites is unchanged and axon elongation is normal and mice show no evidence of white matter disease
(J:302256)
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• cortical neurons grown on poly-D-lysine-coated plates show only marginal changes in morphology during the first 3 days and show reduced rate of formation of ganglial networks at 96 hours, indicating reduced growth of cortical neurons
• cortical neurons fail to respond to EGF-induced growth and maturation
• however, proliferation rate of cortical neurons in response to EGF is similar to wild-type neurons
|
• axonal termini of cortical neurons appear as fringed or feathered with minute processes
• however, axonal length of cortical neurons does not differ from wild-type neurons
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Mast syndrome | DOID:0060245 |
OMIM:248900 |
J:302256 |