muscle
• in quadriceps muscles
• increased variation in fiber size
• very few centrally located nuclei
|
• grouped atrophy in quadriceps muscles
• extensive, at 3 months of age
|
• severe, at 3 months of age
|
behavior/neurological
• reduced muscle strength from 4 weeks of age in all mice
|
nervous system
• significantly reduced number of choline-acetyltransferase-positive motor neurons in spinal cord
|
growth/size/body
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
amyotrophic lateral sclerosis | DOID:332 |
OMIM:PS105400 |
J:241305 |