Analysis Tools
growth/size/body
weight loss
(
J:134095
)
|
• at end-stage, most mice display severe weight loss (>30%)
|
behavior/neurological
|
• at end-stage, severe locomotor deficits are exhibited
|
|
• at end-stage, loss of ability to hang in hanging wire test
|
|
• loss of grip strength at end-stage of disease
|
nervous system
|
• accumulation of argyrophilic neuronal debris is seen throughout medullary, pontine, and mesencephalic reticular formation up to zona incerta
|
|
• animals not displaying motor abnormalities show ubiquinated neurites in spinal cord
|
|
• in mice at end-stage, denervation is observed at neuromuscular synapses
|
|
• appears restricted to brainstem and spinal cord
|
|
• accumulation of argyrophilic neuronal debris is seen in spinal cord of mice at end-stage of disease; similar amounts are seen between left and right, and lumbar and cervical segments
|
|
• loss of motor neurons is detected at disease end-stage
|
muscle
|
• exhibited by most mice (67%) before 2 years of age; onset is observed at 539 days to more than 730 days with end stage reached at 591 to greater than 730 days
|
cellular
| N |
• mice do not develop mitochondrial swelling and vacuolization like G1 or G1del mutants
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyotrophic lateral sclerosis type 1 | DOID:0060193 |
OMIM:105400 |
J:134095 | |
