nervous system
• tau-positive spheroidal inclusions in spinal cord neurons are visible by 1 month of age
• size and number increases up to 6-9 months of age, but decreases in number by 12 months of age
• inclusions are the size of medium to large spinal cord neurons and some appear to arise within the proximal axons of spinal cord neurons
• spinal cord inclusions are most frequent at the grey-white matter junction
• spinal cord inclusions in line 43 are larger and more abundant than in line 7
• tau-positive inclusions appear in cortical neurons and brainstem by 6 months of age
• inclusions in cortical neurons occur in proximal axons or somadendritic domains of neurons
• inclusions are visible in pontine neurons by 1 month of age
• tau deposits become increasingly insoluble with age
|
astrocytosis
(
J:61052
)
• detectable after one month of age
|
• in addition to inclusions, vacuolar lesions are observed in older mice
|
• spinal cord ventral root contains irregularly shaped axons
• by 12 months of age endoneurial space in ventral root axons appears to increase
|
• 20% decrease in number of ventral root axons by 12 months of age
• significant reduction in microtubule density by 12 months of age, although neurofilament density is unchanged
|
• fast axonal transport of proteins is retarded as compared to wildtype
|
behavior/neurological
limb grasping
(
J:61052
)
• mice retract hindlimbs when lifted by the tail
|
• impaired ability to stand on slanted surface
|
growth/size/body
• mice weigh 30-40% less than normal littermates
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Alzheimer's disease | DOID:10652 | J:61052 | ||
frontotemporal dementia | DOID:9255 |
OMIM:600274 |
J:61052 |