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*D177N*M128V)A21Rchi mutation
(1 available)
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mortality/aging
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• mice die from neurological disease with a mean lifespan of around 284 days
• the length of illness (mean day of onset until death) is around 130 days
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behavior/neurological
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• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail
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• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset around 144 days
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• mice develop ataxia with extension of hindlimbs and the unbalanced posture with an age of onset around 144 days
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skeleton
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• kyphosis occurs with the onset of ataxia
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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*D177N*M128V)A21Rchi mutation
(1 available)
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mortality/aging
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• mice die from neurological disease with a mean lifespan of around 704 days
• the length of illness (mean day of onset until death) is around 293 days
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behavior/neurological
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• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail
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• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset around 452 days
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• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset around 452 days
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skeleton
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• kyphosis occurs with the onset of ataxia
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|
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp*D177N*M128V)A21Rchi mutation
(1 available)
|
|
|
mortality/aging
|
|
• mice die from neurological disease with a mean lifespan of 284 days
• the length of illness (mean day of onset until death) is 130 days
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behavior/neurological
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• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail
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|
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• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset at 144 days
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• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset at 144 days
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nervous system
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• protease resistant Prnp deposits are found in many parts of the brain in mice suffering from neurological disease
• Prnp deposits ranged from diffuse synaptic-type depositions prominent in the hippocampus to plaque-like depositions in the reticular thalamic nucleus, the corpus callosum, the external and internal capsule, the cingulated cortex, and the anterior commissure nuclei
• depositions do not contain amyloid fibrils
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• hypertrophy and proliferation of astrocytes are prominent adjacent to Prnp depositions
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skeleton
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• kyphosis occurs with the onset of ataxia
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|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp*D177N*M128V)A21Rchi mutation
(1 available)
|
|
|
mortality/aging
|
|
• mice die from neurological disease with a mean lifespan of 704 days
• the length of illness (mean day of onset until death) is 293 days
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behavior/neurological
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• mice younger than 320 days (i.e. before ataxia occurs) make significantly more errors in an eight-arm radial maze over 10 training days than controls
• latency to complete the test is similar to controls demonstrating that the deficit is unrelated to motor abnormalities
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• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail
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• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset at 452 days
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• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset at 452 days
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• the mean latency to fall for mice in a rotarod test is 100 seconds less than for controls
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• mice spend about 10-fold less time in REM sleep than controls during both light and dark phases
• mice also spend less time in non-REM sleep during the dark phase too
• however, the total time sleeping is not significantly different
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nervous system
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• protease resistant Prnp deposits are found in many parts of the brain in mice suffering from neurological disease
• Prnp deposits ranged from diffuse synaptic-type depositions prominent in the hippocampus to plaque-like depositions in the reticular thalamic nucleus, the corpus callosum, the external and internal capsule, the cingulated cortex, and the anterior commissure nuclei
• depositions do not contain amyloid fibrils
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• the endoplasmic reticulum of granule neurons is swollen and fragmented in mice prior to the onset of disease
• the transgenic Prnp protein localizes in the ER of these cells instead of in the plasma membrane like the endogenous protein
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• hypertrophy and proliferation of astrocytes are prominent adjacent to Prnp depositions
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• EEG activity is characterized by bursts of polyphasic complexes lasting from 0.3 to 4.6 seconds
• the power frequency spectrum of the polyphasic complexes is 7-8 Hz
• polyphasic complexes occur during wake or sleep periods with similar frequency
• most mice also have abnormal ''sawtooth'' waves with a frequency to power spectrum that peak around 4 Hz
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skeleton
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• kyphosis occurs with the onset of ataxia
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Analysis Tools