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(Prnp*P101L)2866Sbp mutation
(0 available)
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mortality/aging
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• mutants die within about 3 days following clinical signs of neurological disease
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nervous system
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• brains of ill mutants contain abundant prion protein amyloid plaques; prion protein deposits are not resistant to proteinase K digestion
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• mean age for development of neurologic disease is 146 +/- 2 days
• mean duration of illness (time from appearance of clinical signs to death) is reduced from 17 +/- 3 days in single Tg(Prnp*P101L)2866Sbp mice to 3 +/-1 days in double mutants
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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*P101L)2866Sbp mutation
(0 available)
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nervous system
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• mean age for development of neurologic disease is 85 +/- 2 days
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
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mortality/aging
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• mice die on average about 17 days following development of clinical signs of neurological disease
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nervous system
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• occasional prion protein plaques are seen in brains of a few ill mutants
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• reactive astrocytic gliosis
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• sciatic nerve shows regional loss of axons and active Wallerian degeneration
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• sciatic nerve shows regional loss of axons
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• mean age of neurologic disease onset is 220 +/- 10 days
• mean duration of illness (time from appearance of clinical signs to death) is 17 +/- 3 days
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• sciatic nerve shows active Wallerian degeneration
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muscle
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• subsarcolemmal accumulation of mitochondria and abnormalities in distribution of the sarcoplasmic reticulum
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• skeletal muscles of aged mutants exhibit neuropathic and myopathic changes around 200 days of age
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• occasionally, degenerating skeletal muscle fibers are seen with rare fibers undergoing phagocytoisis
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• skeletal muscle from clinically ill mutants shows type I fiber predominance and grouping indicative of neurogenic rearrangement of muscle fiber typesskeletal muscle from clinically ill mutants shows type I fiber predominance and grouping indicative of neurogenic rearrangement of muscle fiber types
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homeostasis/metabolism
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