behavior/neurological
abnormal gait
(
J:69545
)
• slightly abnormal gait
|
nervous system
• 26% of axons within the sciatic nerve exhibit very thin or absent myelin
• mice fed tetracycline at 8 weeks of age to turn off transgene expression show myelination of non-myelinated fibers within 1 week of tetracycline feeding, however myelin sheaths of these fibers are still very thin by 2 weeks of feeding
• mice fed tetracycline at 8 weeks of age for 4-12 weeks show a dramatic decrease in the percentage of very thinly myelinated fibers, however the myelin sill has not reached normal thickness
|
• a proportion of axons in 16 week old mice (particularly medium and smaller sized axons) have very thin or no myelin
• mice administered tetracycline from at least a week before birth to turn off transgene expression exhibit nearly normal myelin thickness at 16 weeks of age and only about 1% of axons show very thin or absent myelin
• mice fed tetracycline from before birth to 8 weeks of age develop nearly normal mature myelin
|
demyelination
(
J:69545
)
• mice fed tetracycline from before birth to 8 weeks of age at which age tetracycline was removed to turn on transgene expression show some demyelination such that after 8 weeks of expression, 9% of fibers are demyelinated
|
• motor nerve conduction velocity in the sciatic/tibial nerves is slightly reduced
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Charcot-Marie-Tooth disease type 1A | DOID:0110148 |
OMIM:118220 |
J:69545 |