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Phenotypes Associated with This Genotype
Genotype
MGI:3836993
Allelic
Composition
Tg(Prnp*D177N*M128V)A21Rchi/0
Genetic
Background
involves: C57BL/6 * CBA
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No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
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

behavior/neurological
• 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
• mice suffering form neurological disease tightly clasp hindlimbs upon lifting by tail
• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset at 452 days
• mice develop ataxia with extension of hindlimbs and unbalanced posture with an age of onset at 452 days
• the mean latency to fall for mice in a rotarod test is 100 seconds less than for controls
• 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

nervous system
• 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
• 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
• hypertrophy and proliferation of astrocytes are prominent adjacent to Prnp depositions
• 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

skeleton
• kyphosis occurs with the onset of ataxia

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Creutzfeldt-Jakob disease DOID:11949 OMIM:123400
J:142098


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
03/13/2019
MGI 6.13
The Jackson Laboratory