behavior/neurological
muscle
• muscle atrophy in the hindlimbs
|
nervous system
• mice develop a neuromuscular disorder between 4-8 weeks of age
|
• sciatic nerve shows demyelinating neuropathy
• onion bulbs, signifying myelin destruction followed by attempts to remyelinate, are present at 6 months of age and increase in number with age
|
• hypomyelination with occasional nude axons
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Charcot-Marie-Tooth disease type 1B | DOID:0110152 |
OMIM:118200 |
J:105751 |