mortality/aging
• some deaths among adolescent mice but most have normal life spans
|
behavior/neurological
• first noticeable at 2 weeks of age
|
• first noticeable at 2 weeks of age
|
• stiff gait
• first noticeable at 2 weeks of age
|
muscle
• highly elevate baseline motor unit activity in muscles of both forelimb and hind limb
• motor unit activity reduced by diazepam which enhances GABAergic transmission
|
nervous system
• oval shaped aggregates of vescicular structures with 2-layer membranes in the gray matter of brainstem and spinal cord
• inclusions found in neuronal processes only, possibly in dendrites
• become detectable at 2-4 weeks of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Stiff-Person syndrome | DOID:13366 |
OMIM:184850 |
J:105231 |