nervous system
• develop convulsions by 1.5 months of age
|
astrocytosis
(
J:142650
)
• in the cerebellum and C1-C5 regions of the spinal cord at 6 months of age
|
• optic nerves display areas of myelinolysis and thinly myelinated axons
• gene dosage correlated decrease in myelinated fiber density and myelin thickness
|
• show clinical signs of demyelination (tail droop, shaky gait, head shaking, convulsions, impaired righting response, reduced physical activity) by 1.5 months of age
• signs increase in severity with age but not as rapidly as in homozygous mice
|
behavior/neurological
• display proprioceptive positioning defects at 4 months of age
|
• becomes impaired by 1.5 months of age
|
• at 4 months of age
|
• at 4 months of age
|
• develop tail droop by 1.5 months of age
|
head shaking
(
J:142650
)
• develops by 1.5 months of age
|
• develop a shaky gait by 1.5 months of age
|
• develop convulsions by 1.5 months of age
|
homeostasis/metabolism
• increase in the levels in the brain by 4 months of age
|
immune system
• increase in the levels in the brain by 4 months of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
multiple sclerosis | DOID:2377 |
OMIM:612594 OMIM:612595 OMIM:612596 |
J:142650 |