Analysis Tools
mortality/aging
 |
• mice begin to die from the 4th week of age and less than 20% survive beyond the 6th week and all die by 12 weeks
|
behavior/neurological
|
|
• tremor followed by tonic seizures during the 4th-5th week of age, which increases in frequency and severity
|
|
|
• mice spend less time on the rotarod
|
|
|
• mice exhibit an increase in missteps in the foot slip test
|
|
|
• tremor followed by tonic seizures during the 4th-5th week of age, which increases in frequency and severity
|
hematopoietic system
microgliosis
(
J:219594
)
|
|
• increase in activation of microglia in the optic nerve
|
immune system
microgliosis
(
J:219594
)
|
|
• increase in activation of microglia in the optic nerve
|
|
|
• inflammation in the spinal cord
|
|
|
• inflammation in the optic nerve
|
cellular
|
|
• increase in oligodendrocyte apoptosis
|
nervous system
|
|
• tremor followed by tonic seizures during the 4th-5th week of age, which increases in frequency and severity
|
|
|
• increase in oligodendrocyte apoptosis
|
microgliosis
(
J:219594
)
|
|
• increase in activation of microglia in the optic nerve
|
|
|
• inflammation in the spinal cord
|
|
|
• inflammation in the optic nerve
|
astrocytosis
(
J:219594
)
|
|
|
|
|
• oligodendrocytes are gap junction deficient
|
|
|
• axonal degeneration of oligodendrocytes in the spinal cord funiculi and the optic nerve
|
|
|
• severe demyelination of oligodendrocytes in the spinal cord funiculi and the optic nerve
|
|
|
• some large axons in the anterior and posterior funiculi of the spinal cord are thinly myelinated
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| hypomyelinating leukodystrophy 2 | DOID:0060787 |
OMIM:608804 |
J:219594 | |
