Analysis Tools
mortality/aging
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• median survival of 5.5 months compared to 26 months in wild-type
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growth/size/body
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• mice fail to gain weight normally from 1 month of age on
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behavior/neurological
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• mice exhibit atypical patterns of exploration characterized by frequent changes of direction and spend more time in the center of the open field
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• mice eat more and spend more time visiting food pellets at 5 months of age than controls
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• mice exhibit a lower preference for 2% and 4% sucrose solution than controls
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• mice spend more time in the center of the open field
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• mice exhibit repetitive face and ear cleaning
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• 70% of mice show an altered righting reflex when placed on the back between 4 and 6 months of age
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• mice are unable to extend their hindlimbs when held by the tail as early as 3 months of age
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• on the accelerating rotarod, 3 month old mice show a 2/3 reduction of motor capacities
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• mice develop twitches
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head tossing
(
J:237873
)
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• mice exhibit recurrent repetitive head flicking
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• mice progressively develop motor dysfunction and gait alterations
• mice show a change in footprint pattern, with a 60%, 49% and 60% decrease in footstep length, width, and diagonal, respectively
• mice exhibit an exaggerated spacing of hindlimb feet position and paw placement relative to the body axis, with changes becoming more pronounced with age
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• mice show a 60% decrease in footstep length
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• mice make fewer rearings but rearings occur mostly in the center of the arena
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• mice eventually show progressive hindlimb paralysis associated with a severely stilted gait
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• all mice exhibit stereotypical behavior in their home cage at 4-6 months of age, including upright scrabbling, repetitive burying and relentless chewing of a piece of food
• mice exhibit recurrent repetitive head flicking, kangaroo-like position with mouse in up-right position sitting on its rear, and repetitive face and ear cleaning
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• mice exhibit upright scrabbling (rearing along a wall or in a corner and appearing to run or climb in place) and recurrent kangaroo-like position with mouse in up-right position sitting on its rear
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• mice show a decrease of social interaction with their cage mates
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muscle
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• electromyography of resting activity in the gastrocnemius muscle shows abnormal spontaneous activity with fibrillation potentials when paralysis occurs and numerous fasciculations are seen, suggesting muscle denervation
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• electromyography of resting activity in the gastrocnemius muscles shows numerous fasciculations
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nervous system
astrocytosis
(
J:237873
)
|
• 6 month old mice exhibit astrogliosis in the cortex and spinal cord
• astrocytic activation is more pronounced than microglial activation
|
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• number of denervated or partially denervated neuromuscular junctions is 70% higher at 12 months than in wild-type littermates
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• mice develop age-dependent cytoplasmic inclusions immunoreactive for p62 and ubiquitin
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• the proportion of large myelinated axons in the sciatic nerve is decreased while the number of small caliber myelinated axons is increased
• however, mice show normal numbers of motor neurons in the ventral horn of the spinal cord
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| frontotemporal dementia | DOID:9255 |
OMIM:600274 |
J:237873 | |
| frontotemporal dementia and/or amyotrophic lateral sclerosis 7 | DOID:0111227 |
OMIM:600795 |
J:237873 | |
