Analysis Tools
hematopoietic system
microgliosis
(
J:239141
)
|
• mild gliosis is observed in anterior horn of spinal cord in young mice
• moderate gliosis is observed in white matter tracts
• mild increase in CD68+ microgliosis in layer V
|
nervous system
microgliosis
(
J:239141
)
|
• mild gliosis is observed in anterior horn of spinal cord in young mice
• moderate gliosis is observed in white matter tracts
• mild increase in CD68+ microgliosis in layer V
|
|
• loss of alpha motor neurons in layer V region of the cortex
• 46% loss of large caliber alpha-motor axons in L5 nerve root at 8 weeks of age
• decreased numbers of pyramidal neurons in layer V
• astrogliosis is observed in layer V of cortex of young animals
• mild increase in CD68+ microgliosis in layer V
|
|
• small cytoplasmic p62+ and TARDBP inclusions, as well as occasional ubiquitin inclusions are observed in motor and somatosensory cortices of 8 week old mice
|
|
• small cytoplasmic p62+ and TARDBP inclusions, as well as occasional ubiquitin inclusions are observed in motor and somatosensory cortices of 8 week old mice
|
astrocytosis
(
J:239141
)
|
• astrogliosis is observed in layer V of cortex of young animals
• astrogliosis is observed in anterior horn of spinal cord in young mice
|
|
• 46% loss of large caliber alpha-motor axons in L5 nerve root at 8 weeks of age
• axon degeneration is characterized by vacuolization and myelin defects
|
|
• 70% loss of alpha motor neurons in anterior horn of lumbar spinal cord
• loss of alpha motor neurons in layer V region of the cortex
• decreased numbers of pyramidal neurons in layer V
|
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• decreased numbers of pyramidal neurons in layer V
|
|
• 60% reduction in intact neuromuscular junctions in 8 week old mice
• reduction in NMJ area in 8 week old mice
|
|
• frequent globular cytoplasmic inclusions are observed in spinal cord at 8 weeks of age
• a small number of ubiquitin and p62+ inclusions are observed in spinal cord of 8 week old mice
• small cytoplasmic p62+ and TARDBP inclusions, as well as occasional ubiquitin inclusions are observed in motor and somatosensory cortices of 8 week old mice
|
|
• multiple small globular inclusions in neuropil of ventral horns
• nuclear aggregation of endogenous TDP-43 in 8 week old mice
|
|
• astrogliosis and mild gliosis in anterior horn of spinal cord in young mice
• frequent globular cytoplasmic inclusions are observed in spinal cord at 8 weeks of age
• multiple small globular inclusions in neuropil of ventral horns
• moderate gliosis is observed in white matter tracts
|
|
• 70% loss of alpha motor neurons in anterior horn of lumbar spinal cord
|
|
• progressive motor and cortical neuron degeneration
|
immune system
microgliosis
(
J:239141
)
|
• mild gliosis is observed in anterior horn of spinal cord in young mice
• moderate gliosis is observed in white matter tracts
• mild increase in CD68+ microgliosis in layer V
|
mortality/aging
|
• average survival time is 64 +/- 4 days
|
muscle
|
• muscle fiber atrophy
|
behavior/neurological
|
• failure to splay limbs normally when lifted by tail
|
|
• rapid decline in motor function from 3 weeks of age
• decline in performance on rotarod beginning at 5 weeks of age as compared to controls
|
|
• severely stilted gait
|
|
• mice lack the ability to raise pelvis from ground
• progressive, severe hind-limb paralysis beginning at 5-6 weeks of age
|
digestive/alimentary system
| N |
• no intestinal blockage or megacolon is observed
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyotrophic lateral sclerosis type 10 | DOID:0060201 |
OMIM:612069 |
J:239141 | |
