Mouse Genome Informatics
tg
    Tg(PLP1*)4Rsj/0
involves: CD-1
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• death by 8-10 months of age (J:24069)

nervous system
• seizure like state when picked up or otherwise disturbed
• lymphocytic infiltration is observed in the parenchyma in areas of degenerating myelin
• brain volume of 8 month old mutants is only 85% of brain volume in controls
• a 2 fold increase at 3 months and extensive (30 times a 3 month wild-type) in 10 month old mice
• areas of absent, thin, or degraded myelin in the optic nerve
• areas of thin myelination in the optic nerve (J:24069)
• approximately 60% of the axons are thinly myelinated in 8 month old mice (J:161344)
• nude and degenerated axons are evident in 8 month old mice (J:161344)
• partially degraded sheaths are evident in optic nerve (J:24069)
• in optic nerve of 8 month old mice, extensive unmyelinated areas (J:24069)
• at 7 months of age, but not at 2 months of age, the amount of myelin in whole brain homogenates is significantly reduced (J:24069)
• optic nerves show denuded axons in 8 month old mice (J:161344)
• whole brains show reduced levels of myelin at 10 months of age (J:161344)
• onset of demyelination in the brain at 5 months of age that gets progressively worse over time (J:171534)

behavior/neurological
• head and neck tremble when raised (J:24069)
• spastic tremor at 3 months of age (J:171534)
• unsteadiness that progresses with age
• mice wobble with each step at 6 months
• observed at 3 months of age
• gait abnormalities at 3 months of age
• seizure like state when picked up or otherwise disturbed

immune system
• lymphocytic infiltration is observed in the parenchyma in areas of degenerating myelin

vision/eye
• pattern electrotetinogram (PERG) waveforms show a progressive reduction in amplitude with age, indicating dysfunction of the retinal ganglion cells and of vision
• the initial PERG amplitude decrease occurs before the onset of clinical symptoms or demyelination

Mouse Models of Human Disease
OMIM IDRef(s)
Multiple Sclerosis, Susceptibility To; MS 126200 J:161344 , J:171534