mortality/aging
• mean age of death is 372 +/- 78 days
|
behavior/neurological
• mutants first show signs of hindlimb paraparesis and relatively symmetrical forelimb weakness (hemiparesis)
|
paraparesis
(
J:97932
)
nervous system
• eosinophilic cytoplasmic inclusions are seen in the motor neurons that remain; inclusions resemble Lewy body-like hyaline inclusions, with the halo of inclusions composed of neurofilamentous structure and the core of granule-associated fibrils
|
• mean age of onset of motor neuron disease symptoms is 337 +/- 101 days
|
• loss of anterior horn cells in the spinal cord, predominantly in the lower spinal cord
|
muscle
• muscle atrophy is seen at the end stage of disease
|
• some mutants exhibit muscle cramps after exercise as a first symptom of disease
|
skeleton
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
amyotrophic lateral sclerosis type 1 | DOID:0060193 |
OMIM:105400 |
J:97932 |