Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nefltm1Jpj mutation
(0 available);
any
Nefl mutation
(17 available)
Tg(SOD1*G85R)148Dwc mutation
(1 available)
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nervous system
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• at 12 months of age, no axon loss or degeneration in motor nerves
• mice start out with a 13% reduction of motor neurons
• further degeneration is delayed but ultimately reaches the same level of motor axon loss
• loss of axons in sensory neurons is increased
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• slower development of ALS symptoms than in the presence of the transgene alone
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G85R)148Dwc mutation
(1 available)
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nervous system
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• inclusions have a dense core and a clear peripheral halo
• at 6 months of age round Lewy body-like inclusions are visible in astrocytes
• number of inclusions increases with age
• core consists of heterogeneous mass of short filamentous material covered with small granules
• periphery has a less dense and, in some cases, linear array of filaments
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• abnormalities are evident in ventral motor neurons, small neurons of the central canal and interneurons of the dorsal horns, however, no abnormalities are observed in cortical or subcortical structures
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• prior to onset of disease a small number of motor neurons exhibit a few diffuse aggregates that are immunoreactive for SOD1 and ubiquitin
• aggregates progress to rounded Lewy body-like or irregular inclusions in cell bodies
• end-stage transgenics exhibit large inclusions in a few neurons in cell bodies and axonal processes
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• loss of motor neurons in ventral horn of spinal cord
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• at 6.5 months a small number of large ventral motor neuron axons exhibit degeneration, although the loss is not significant
• at 8 months (disease onset) 25% of large motor axons are absent , of the remaining axons, 10% are undergoing degeneration and within another two weeks 66% are absent
small caliber axons are not affected
• at 8 months 2.5% of large sensory axons are absent
• among large axons, 7.5% of dorsal root axons are absent at end stage
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behavior/neurological
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• weakened grip strength, the first indication of phenotype, is observed by 8 months and spreads rapidly to other limbs
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• complete paralysis occurs two weeks after onset of weakened grip strength
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• hindlimb paralysis is first observed at 8-10 months
• paralysis occurs 2-3 days after appearance of hindlimb weakness
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muscle