mortality/aging
• average lifespan of mutants showing amyotrophic lateral sclerosis-like phenotypes is 10 months
|
growth/size/body
• average weight is slightly lower at early birth than in controls and this difference becomes more pronounced with age, with a significant weight difference after 8 weeks of age
|
weight loss
(
J:190254
)
• while mutants show a peak of weight gain during 90-100 days of age, soon after this time, they begin to show weight loss
|
behavior/neurological
• abnormal hind limb clasping is seen after 13 weeks of age
|
• mutants show a deficiency in the rotarod test after 13 weeks of age
|
immune system
• microglia activation is seen in the lateroventral lumbar spinal cord
|
muscle
• more males than females develop amyotrophic lateral sclerosis-like phenotypes with a male/female ratio of 3:1
|
nervous system
• microglia activation is seen in the lateroventral lumbar spinal cord
|
astrocytosis
(
J:190254
)
• in the spinal cord
|
• accumulation of ubiquitinated proteins in the spinal cord motor neurons at 20 weeks of age
|
• progressive loss of motor neurons, with a 10% decrease of spinal cord motor neurons at 10 weeks of age and a large loss of ChAT-positive motor neurons at 20 weeks of age
• 46% and 25% reduction in alpha and gamma motor neurons, respectively, in the lumbar regions of the spinal cord at 20 weeks of age
• more males than females develop amyotrophic lateral sclerosis-like phenotypes with a male/female ratio of 3:1
|
• motor neuron loss, reactive astrocytosis, microglia activation and accumulation of polyubiquitinated proteins in the ventral horn
|
skeleton
• mutants exhibit kyphosis beginning at 20 weeks of age which becomes severe at 24 weeks
|
hematopoietic system
• microglia activation is seen in the lateroventral lumbar spinal cord
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
amyotrophic lateral sclerosis type 10 | DOID:0060201 |
OMIM:612069 |
J:190254 |