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Phenotypes Associated with This Genotype
Genotype
MGI:5546408
Allelic
Composition
Prnptm3Lnq/Prnptm3Lnq
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prnptm3Lnq mutation (0 available); any Prnp mutation (88 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Neuropathologic processes of Prnptm3Lnq/Prnptm3Lnq mice

mortality/aging
• some mice become terminally ill by 14 months of age

behavior/neurological
• performance on a rotarod improves in repeated trials but lags far behind controls
• only half as efficient in the ability to remove a burrowing matrix from a tube
• in an automated mouse behavioral analysis assay, "turn" (a measure of body twisting behavior) and "cuddled hang" (time spent hanging from the ceiling) are strongly reduced
• in an automated mouse behavioral analysis assay, might generally spend less time doing physically demanding behaviors and more time in resting-related behaviors

nervous system
• signs of programmed cell death are seen in the thalamus
• markers of proliferating neurons are detected in the superior colliculus
• numerous proteinase K-resistant prion protein aggregates are present
• prominent spongiform degeneration in the CA1 region of the hippocampus, specifically in the synapse-rich area of the molecular layer
• small patches of proteinase K-resistant prion protein aggregates are present
• numerous proteinase K-resistant prion protein aggregates are present
• reactive gliosis in the hippocampi and deep cerebellar white matter but not in the thalamus
• abnormally large H2A.X positive puncta are present in striatal neurons
• prominent spongiform degeneration in the CA1 region of the hippocampus, specifically in the synapse-rich area of the molecular layer
• the disease is transmissible to other strains of mice following exposure to brain homogenates from ill mice

vision/eye
• proteinase K-resistant prion protein aggregates are present in a synapse-rich region of the retina

cellular
• signs of programmed cell death are seen in the thalamus
• markers of proliferating neurons are detected in the superior colliculus

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Creutzfeldt-Jakob disease DOID:11949 OMIM:123400
J:200974


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/05/2019
MGI 6.13
The Jackson Laboratory