Analysis Tools
behavior/neurological
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• females exhibit a sensory deficit in response to a low threshold mechanical stimulus
• however, females do not show deficits toward a high-threshold mechanical stimulus
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• mice show impaired performance on the accelerating rotarod, with females showing earlier deficits (23 weeks) than males (67 weeks)
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• mice exhibit reduced grip strength from 6 weeks of age
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• mice develop gait abnormalities with age
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• ability to walk down a vertical wire grate (negative geotaxis) is impaired
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• females exhibit a sensory deficit in response noxious heat stimulus
• however, females do not show deficits toward a noxious cold stimulus (cold plate)
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growth/size/body
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• mice show less fat mass at 52 weeks of age
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• reduction in body weight from 4 weeks of age
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hematopoietic system
microgliosis
(
J:219360
)
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• microgliosis is seen in the lumbar spinal cord at 15 weeks of age and increases further at 52 weeks of age
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immune system
microgliosis
(
J:219360
)
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• microgliosis is seen in the lumbar spinal cord at 15 weeks of age and increases further at 52 weeks of age
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• liver at autopsy shows moderate parenchymal inflammation
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liver/biliary system
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• liver at autopsy shows moderate parenchymal inflammation
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• liver at autopsy shows diffuse nuclear anisocytosis, moderate parenchymal inflammation and patchy fatty change
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• mice exhibit more liver tumors at autopsy than wild-type mice
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mortality/aging
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• fewer than the expected numbers of homozygotes are obtained
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• shorter survival, with males having a significantly reduced lifespan compared to females (495 days vs. 588 days, respectively)
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muscle
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• mice exhibit changes in the histochemical phenotype of fast twitch muscles indicative of an oxidative phenotype that is more characteristic of slow twitch, type I muscle fibers
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• progressive loss in muscle strength between 15 and 52 weeks of age, with weaker tibialis anterior muscle at 15 weeks that declines further at 52 weeks, and a reduction in muscle force of EDL by about 35% in 52 week old females
• the fatigue index of EDL is normal at 15 weeks of age but is increased by 30% at 52 weeks of age
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nervous system
microgliosis
(
J:219360
)
|
• microgliosis is seen in the lumbar spinal cord at 15 weeks of age and increases further at 52 weeks of age
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astrocytosis
(
J:219360
)
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• astrogliosis is seen in the lumbar spinal cord at 15 weeks of age and increases further at 52 weeks of age
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• mice exhibit an increase in denervated endplate neuromuscular junctions in the extensor digitorum longus (EDL) at 52, but not 15, weeks of age indicating distal progressive denervation of the EDL muscle
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• mice show signs of cellular degeneration of corticospinal motor neurons in layer V of the motor cortex at 29 weeks of age
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• corticospinal motor neuron numbers are normal at 15 weeks of age but are reduced by 22% at 29 weeks of age
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• mice develop lower motor neuron 9in the lumbar spinal cord) degeneration between 6 and 15 weeks of age, showing a 23% reduction in the number of lower motor neurons at 15 weeks of age, which remains stable at 52 weeks of age
• mice develop upper motor neuron degeneration between 15 and 29 weeks of age, with a 22% reduction in corticospinal motor neuron numbers at 29 weeks
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skeleton
neoplasm
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• mice exhibit more liver tumors at autopsy than wild-type mice
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adipose tissue
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• mice show less fat mass at 52 weeks of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyotrophic lateral sclerosis type 1 | DOID:0060193 |
OMIM:105400 |
J:219360 | |
