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)46Jcol mutation
(0 available)
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nervous system
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• both slow and medium after hyperpolarization (AHP) responses are decreased compared to in wild-type mice (slow AHP, -0.97+/-0.13 mV compared to -0.3+/-0.05 mV in wild-type mice; medium AHP, -1.8+/-0.15 mV to -1.35+/-0.1 mV in wild-type mice)
• however, resting potentials, input resistances, and action potential thresholds and amplitudes are normal
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immune system
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• no mice develop scrapie for up to 400 days post-infection with no evidence of scrapie or accumulation of PrnpSc in one mouse that was culled due to intercurrent illness
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behavior/neurological
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N |
• mice are indistinguishable from wild-type mice with normal gait, posture, motor control, coordination, autonomic function, general excitability and aggression at time points when cre is expressed, 3 to 5 months and 6 to 15 months
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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)46Jcol mutation
(0 available)
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nervous system
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N |
• at 18 months of age mice are indistinguishable from wild-type mice with no evidence of neuropathology
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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:
Scn3atm1.1Jwo mutation
(1 available);
any
Scn3a mutation
(110 available)
Tg(Nefh-cre)22Jcol mutation
(0 available)
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behavior/neurological
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N |
• mice exhibit normal neuropathic pain behavior
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Analysis Tools