Analysis Tools
mortality/aging
|
• by 10 to 13 weeks (average survival 82 days)
|
behavior/neurological
|
• from 4 weeks, mice exhibit rapid decline in motor function
|
|
• by 8 weeks
|
|
• from 4 weeks
|
|
• slightly stilted at 4 weeks due to hind limb dysfunction
|
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• by 8 weeks, mice travel less distance in an open field compared with wild-type mice
|
|
• progressive by 8 weeks
|
nervous system
microgliosis
(
J:189360
)
|
• in the anterior horn and white matter tracts of the spinal cord
|
astrocytosis
(
J:189360
)
|
• in the anterior horn of the spinal cord
|
|
• at end-stage, mice exhibit numerous ring-like perinuclear inclusions in layer V neurons of the motor and somatosensory cortices, the insular cortex, the neostriatum and the Purkinje cells of the cerebellum compared with wild-type mice
• however, mice do not exhibit ubiquitinated FTLD-FUS-like inclusions
|
|
• mice exhibit fewer motor units in muscles compared with wild-type mice
|
|
• ALS-like pathology
|
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• in the anterior horn and white matter tracts of the spinal cord
|
muscle
|
• mice exhibit a loss of glycolytic fast-twitch muscle fibers compared with wild-type mice
|
|
• at end-stage
|
|
• mice exhibit increased fatigue resistance compared with wild-type mice
|
|
• mice exhibit weaker twitch and tetanic force compared with wild-type mice
|
growth/size/body
|
• from 4 weeks
|
hematopoietic system
microgliosis
(
J:189360
)
|
• in the anterior horn and white matter tracts of the spinal cord
|
immune system
microgliosis
(
J:189360
)
|
• in the anterior horn and white matter tracts of the spinal cord
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyotrophic lateral sclerosis type 6 | DOID:0060198 |
OMIM:608030 |
J:189360 | |
