Analysis Tools
behavior/neurological
|
• rotarod scores start to decrease at 30 weeks of age
|
|
• mice develop motor paralysis starting from the hindlimbs, spreading to the rest of the body; disease onset is on average 239 days in both males and females
• duration of disease is on average 34 and 33 days in males and females, respectively
|
growth/size/body
weight loss
(
J:221350
)
|
• mice begin to lose weight from 29 weeks of age
|
hematopoietic system
microgliosis
(
J:221350
)
|
• 40 week old mice exhibit activation of microglia in spinal cords
|
immune system
microgliosis
(
J:221350
)
|
• 40 week old mice exhibit activation of microglia in spinal cords
|
mortality/aging
|
• shorter lifespan, with average lifespan of 274 and 272 days in males and females, respectively
|
muscle
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• tibialis anterior muscle fibers show typical neuropathic changes (atrophic and infiltrated by inflammatory cells)
|
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• decrease in tibialis anterior muscle weight at 40 weeks of age
|
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• atrophic tibialis anterior muscle fibers
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nervous system
microgliosis
(
J:221350
)
|
• 40 week old mice exhibit activation of microglia in spinal cords
|
astrocytosis
(
J:221350
)
|
• 40 week old mice exhibit activation of astrocytes in spinal cords
|
|
• decrease in spinal cord weight at 40 weeks of age
|
|
• the number of spinal motor neurons is reduced to approximately half of that of wild-type mice
|
|
• SMI-32-positive motor neurons are often pyknotic in the ventral horns of lumbar spinal cords
• the number of spinal motor neurons is reduced to approximately half of that of wild-type mice and transgenic mice with both the H46R and C111S mutations
|
limbs/digits/tail
|
• tibialis anterior muscle fibers show typical neuropathic changes (atrophic and infiltrated by inflammatory cells)
|
|
• decrease in tibialis anterior muscle weight at 40 weeks of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyotrophic lateral sclerosis type 1 | DOID:0060193 |
OMIM:105400 |
J:221350 | |
