Analysis Tools
behavior/neurological
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• in the passive avoidance test, mutants exhibit severe cognitive impairments after 7 months of age and especially in the 11th and 13th months
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• mice show reduction in latency in the accelerating rotarod tests starting at around 42 weeks of age, indicating age-related motor deficits
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nervous system
microgliosis
(
J:195184
)
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• age dependent microgliosis and astrogliosis in the brain and spinal cord; mircrogliosis and astrogliosis is less pronounced than in mice expressing the transgene with the G348C mutation
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astrocytosis
(
J:195184
)
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• age dependent microgliosis and astrogliosis in the brain and spinal cord; mircrogliosis and astrogliosis is less pronounced than in mice expressing the transgene with the G348C mutation
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• 7% increase (an increase of 70 axons) in the number of motor axons with 1-3 um caliber and a 8% decrease (a decrease of 80 axons) in the number of motor axons with 6-9 um caliber in 10 month old mutants
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• mice develop ubiquitinated and TARDBP+ inclusions in the spinal cord, cortex, and hippocampus by 10 months of age
• abnormal accumulation of neurofilament and peripherin proteins in inclusions in large motor neurons and the brain at 10 months of age
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hematopoietic system
microgliosis
(
J:195184
)
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• age dependent microgliosis and astrogliosis in the brain and spinal cord; mircrogliosis and astrogliosis is less pronounced than in mice expressing the transgene with the G348C mutation
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immune system
microgliosis
(
J:195184
)
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• age dependent microgliosis and astrogliosis in the brain and spinal cord; mircrogliosis and astrogliosis is less pronounced than in mice expressing the transgene with the G348C mutation
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyotrophic lateral sclerosis type 10 | DOID:0060201 |
OMIM:612069 |
J:195184 | |
| Grn-related frontotemporal lobar degeneration with Tdp43 inclusions | DOID:0060672 |
OMIM:607485 |
J:195184 | |
