mortality/aging
• mean survival is 349 days
|
behavior/neurological
• progressive paralysis
|
growth/size/body
weight loss
(
J:147156
)
• weight loss begins at 307 days of age
|
nervous system
astrocytosis
(
J:147156
)
• in the anterior horn of the spinal cord at 350 days of age
|
• SOD1-immunoreactive aggregates (of mutant SOD1 homodimers) are seen in motor neuron cells in the anterior horn at 350 days of age
|
• loss of motor neurons at 350 days of age but not at 150 days of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
amyotrophic lateral sclerosis type 1 | DOID:0060193 |
OMIM:105400 |
J:147156 |