Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe mutation
(39 available);
any
Prnp mutation
(150 available)
Tg(Nefh-cre)22Jcol mutation
(0 available)
Tg(Prnp)37Jcol mutation
(0 available)
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behavior/neurological
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• mice display behavioral defects despite normal grip strength and coordination of movement
• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display reduced burrowing (displacing 54+/-7% of pellets compared to 73+/-4% at week 7) that continues to deteriorate into week 9(45+/-6%) but recovers by week 10
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• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories mice exhibit a decrease in exploration of a novel object
• however, following expression of cre at week 8.5 novel object exploration preference is regained by week 12 and persists through week 30
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• at 12 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display decreased activity in an open field compared to Prnptm1Cwe/Prnptm1Cwe Tg(Prnp)37Jcol mice
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nervous system
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• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice exhibit evidence of spongiosis in the hippocampus
• however, there is no evidence of neurodegeneration at 6 weeks post-infection and, with the expression of cre beginning at week 8.5, spongiosis is reversed by week 10
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• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display a 50% reduction in excitatory postsynaptic potential (EPSP) compared to uninfected mice or recovered mice
• however, activation of the cre transgene at 8.5 weeks leads to a recovery of EPSP and long term potentiation is normal
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immune system
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• mice develop clinical symptoms of prion disease but recover, surviving up to 30 weeks post-infection with scrapie strain Rocky Mountain Laboratories, compared to Prnptm1Cwe/Prnptm1Cwe Tg(Prnp)37Jcol mice that develop clinical symptoms of prion disease at 12 weeks post-infection
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm1Cwe mutation
(39 available);
any
Prnp mutation
(150 available)
Tg(Prnp)37Jcol mutation
(0 available)
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mortality/aging
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• mice succumb sooner to scrapies infection at 72+/-5 days compared to 130+/-1 days for wild-type mice
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behavior/neurological
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• mice display behavioral defects despite normal grip strength and coordination of movement
• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display reduced burrowing (displacing 66+/-6% of pellets compared to 73+/-4% at week 7) that continues to deteriorate throughout the course of the infection (21%-28% displacement at week 12)
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• while mice behave normally towards a novel object at 7 weeks post-infection with scrapie strain Rocky Mountain Laboratories, at 8 weeks post-infection mice exhibit an inability to recognize a novel object with no preference towards it that lasts through out the course of infection
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• while mice behave normally towards a novel object at 7 weeks post-infection with scrapie strain Rocky Mountain Laboratories, at 8 weeks post-infection mice exhibit an inability to recognize a novel object with no preference towards it that lasts through out the course of infection
by week 12, novel object preference is reversed possibly due to preference for familiar objects and neophobia
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• by 12 weeks post-infection with scrapie strain Rocky Mountain Laboratories mice exhibit decreased grooming
• however, no other motor signs of prion disease are evident at 12 weeks post-infection
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• at 12 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display increased activity in an open field compared to Prnptm1Cwe/Prnptm1Cwe Tg(Nefh-cre)22Jcol Tg(Prnp)37Jcol mice
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nervous system
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• while after hyperpolarization response is absent in Prnptm1Cwe homozygotes, mice exhibit a slow after hyperpolarization response similar to that observed in wild-type mice
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• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice exhibit evidence of spongiosis in the hippocampus
• however, at 6 weeks post-infection there is no evidence of neurodegeneration
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• at 8 weeks post-infection with scrapie strain Rocky Mountain Laboratories, mice display a 50% reduction in excitatory postsynaptic potential compared to uninfected mice or recovered mice that deteriorates to 22% of uninfected response by week 9
• however, the amplitude of fiber volleys is linearly related to EPSP as in controls indicating a change in presynaptic action potential and long term potentiation is normal
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immune system
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• mice succumb sooner to scrapies infection at 72+/-5 days compared to 130+/-1 days for wild-type mice
(J:74652)
• mice develop clinical symptoms of prion disease at 12 weeks post-infection with scrapie strain Rocky Mountain Laboratories compared to Prnptm1Cwe/Prnptm1Cwe Tg(Nefh-cre)22Jcol Tg(Prnp)37Jcol mice that develop clinical symptoms of prion disease but recover, surviving up to 30 weeks post-infection
(J:126424)
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Analysis Tools