behavior/neurological
N |
• mice exhibit normal spontaneous avoidance
|
• impaired performance in a Morris water maze
• impaired long-term spatial associated learning and fear memory of an averse experience in a step-through passive avoidance task with no increase in latency to avoid the light and step through the dark chamber where they received an electric shock the day before
|
• impaired spatial memory retention in a Morris water maze
• impaired long-term spatial associated learning and fear memory of an averse experience in a step-through passive avoidance task with no increase in latency to avoid the light and step through the dark chamber where they received an electric shock the day before
|
• progressive impairment on an accelerating rotarod (at 24, but not 12, months) and balance beam (at 12 months and worse at 24 months)
|
straub tail
(
J:197168
)
• tail extension
|
• varying from generalized spasms and tremors to generalized clonic activity with atonia and tail extension
• frequent spontaneous epileptic activity, including epileptiform discharge
|
• when treated with pentylenetetrazol
|
• with atonia and tail extension
|
• frequent spontaneous epileptic activity, including epileptiform discharge and a 20-fold increase in the number of interictal spikes
|
nervous system
N |
• mice exhibit no obvious inflammation in the brain
|
• varying from generalized spasms and tremors to generalized clonic activity with atonia and tail extension
• frequent spontaneous epileptic activity, including epileptiform discharge
|
• when treated with pentylenetetrazol
|
• with atonia and tail extension
|
• frequent spontaneous epileptic activity, including epileptiform discharge and a 20-fold increase in the number of interictal spikes
|
• outfoldings, extensions and invaginations originating from the myelin sheath
|
• disintegration and degradation
|
• frequently dying and degenerating at 24 months
|
• in some fibers at 12, but not 24, months
|
• in some fibers
|
muscle
muscle spasm
(
J:197168
)
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
adult-onset autosomal dominant demyelinating leukodystrophy | DOID:0060785 |
OMIM:169500 |
J:197168 |