mortality/aging
• premature death by around P35
|
growth/size/body
• mice fail to gain weight with age
|
behavior/neurological
• mice develop severe movement defects with age
|
• mice exhibit hindlimb clasping by P14
|
• mice develop a stiff tail
|
spasticity
(
J:216408
)
• mice develop hind-limb spasticity
|
nervous system
• brainstem and lumbar spinal regions contain aggregate bodies that are not seen in controls, resembling Bunina bodies which are eosinophilic inclusions
• Bunina-like structures appear to be extracellular
|
skeleton
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
motor neuron disease | DOID:231 | J:216408 |