Mouse Genome Informatics
tg
    Tg(Prnp-MAPT)7Vle/0
involves: C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• inclusions in the somatodendritic compartment of cortical neurons resemble neurofibrillary tangles
• 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 7 are smaller and less abundant than in line 43
• tau-positive intraneuronal aggregates are seen in the brain, but they are smaller and appear later than in the spinal cord
• 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 and in the cerebral cortex at 6 months of age
• tau deposits become increasingly insoluble with age
• 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 at 6 months of age
• 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 in 12 month old mice as compared to wild-type

behavior/neurological
• mice retract hindlimbs when lifted by the tail
• impaired ability to stand on a slanted surface
• progressive motor weakness

growth/size/body
• mice weigh 30-40% less than normal littermates

Mouse Models of Human Disease
OMIM IDRef(s)
Alzheimer Disease; AD 104300 J:61052
Frontotemporal Dementia; FTD 600274 J:61052