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Phenotypes Associated with This Genotype
Genotype
MGI:5525131
Allelic
Composition
Tg(Prnp-ITM2B*)1Ruvi/Tg(Prnp-ITM2B*)1Ruvi
Tg(Prnp-MAPT*P301L)#Ruvi/Tg(Prnp-MAPT*P301L)#Ruvi
Genetic
Background
involves: C3HeB/FeJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
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
• reduction in survival rate beginning around 9 months of age

growth/size/body
• weight loss by 6 months of age to similar extent as in either single mutant mice

behavior/neurological
• abnormal grooming behavior starting around 9 months of age as evident by dull rough coats
• at 12 months of age, 88% of mutants exhibit cupping of the hind paws and bilaterally pulling of the hind paws toward the abdomen when suspended by the tail compared to 33% of wild-type mice
• by 12 months of age, mutants show a hunched back or arched posture when sitting and walking
• 12 month old mutants exhibit an usual gait in which the mouse holds its body near the walking surface and takes wide shortened steps

immune system
• expression analysis indicates neuroinflammation, with activated astrocytes and microglia seen in close proximity of amyloid deposits and neurofibrillary tangles; activated microglia are seen before Danish amyloid deposition and correlates with tau deposition

nervous system
• beginning around 6-7 months of age, mutants show amyloid deposition primarily in leptomeningeal cerebellar vessels and later developing extensive amyloid lesions in the parenchyma and vasculature of the neocortex, hippocampus, and cerebellum
• parenchymal amyloid deposition is most prominent in the CA3 and CA2 regions and the hilus of the hippocampus
• tau deposits are predominately seen in the hippocampus, piriform cortex, brain stem, spinal cord, and the cerebellum
• tau accumulation is enhanced compared to single Tg(Prnp-MAPT*P301L)#Ruvi mice that occurs before extracellular deposition of Danish amyloid plaques
• by 6 months of age, synaptophysin levels are decreased, indicating synaptic degeneration; this is seen earlier and with a more severe loss of synaptophysin than in either single mutant and occurs before the detection of amyloid plaques or tau pathology

homeostasis/metabolism
• beginning around 6-7 months of age, mutants show amyloid deposition primarily in leptomeningeal cerebellar vessels and later developing extensive amyloid lesions in the parenchyma and vasculature of the neocortex, hippocampus, and cerebellum
• parenchymal amyloid deposition is most prominent in the CA3 and CA2 regions and the hilus of the hippocampus

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
cerebral amyloid angiopathy DOID:9246 J:197198


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
10/29/2024
MGI 6.24
The Jackson Laboratory