Mouse Genome Informatics
cx1
    Tg(Prnp-ITM2B*)7Jckr/0
Tg(Thy1-MAPT*P301S)2541Godt/0

B6.Cg-Tg(Prnp-ITM2B*)7Jckr Tg(Thy1-MAPT*P301S)2541Godt
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
• at 12 months, mice exhibit a greater than 50-fold increase in Gallyas+ neurofibrillary pathology compared with Tg(Thy1-MAPT*P301S)2541Godt mice
• at 12 months, mice exhibit an 18-fold increase in neocortex Tau inclusions compared with Tg(Thy1-MAPT*P301S)2541Godt mice


Mouse Genome Informatics
cx2
    Tg(Prnp-ITM2B*)7Jckr/0
Tg(Thy1-APPSw,Thy1-PSEN1*L166P)21Jckr/0

C57BL/6-Tg(Prnp-ITM2B*)7Jckr Tg(Thy1-APPSw,Thy1-PSEN1*L166P)21Jckr
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
• mice exhibit a 68% reduction in neocortical amyloid beta deposition compared with Tg(Thy1-APPSw,Thy1-PSEN1*L166P)21Jckr mice

homeostasis/metabolism
• mice exhibit an accumulation of Dan-amyloid in separate plaques from amyloid beta plaques
• mice exhibit a 68% reduction in neocortical amyloid beta deposition compared with Tg(Thy1-APPSw,Thy1-PSEN1*L166P)21Jckr mice


Mouse Genome Informatics
tg3
    Tg(Prnp-ITM2B*)7Jckr/0
C57BL/6-Tg(Prnp-ITM2B*)7Jckr
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
behavior/neurological
N
• at 6 months, mice exhibit normal behavior (J:159279)
• in Morris water maze, aged mice exhibit increased passive floating and reduced swim speed compared with wild-type mice
• in an open field test, aged mice exhibit reduced activity, preference for the wall zone, reduced vertical activity, and more numerous fecal boli compared with wild-type mice
• in Morris water maze and in an open field tests with aged mice
• at 18 to 20 months, mice take longer to find the platform in the Morris water maze compared with wild-type mice
• during the probe trials, 18 to 20 month old mice tend to have a preference for the training target zone compared with wild-type mice
• during the two probe trials, mice exhibit a minor reduction in searching precision after 24 hours and a total loss of spatial selectivity after 6 days compared with wild-type mice
• in Morris water maze, aged mice exhibit increased passive floating and reduced swim speed compared with wild-type mice
• in an open field test with aged mice
• in an open field test with aged mice

nervous system
• at 18 months, mice exhibit age-dependent, vasculature-associated Dan-amyloid deposition in the amygdala, thalamus, brainstem, and cerebellum unlike wild-type mice
• smaller vessels are often obstructed by depositions
• perivascular plaques often surround a significant portion of the vessel surface
• 18 month old mice exhibit cerebral microhemorrhages unlike in wild-type mice
• 18 month old mice exhibit cerebral microhemorrhages unlike in wild-type mice
• in juxtaposition to amyloid depositions
• neuronal cell bodies in the CA3/4 region of the hippocampus are displaced compared to in wild-type mice
• Dan-amyloid deposition elicits activation of astrocytes throughout the entire brain
• mice exhibit dystrophic neurites unlike wild-type mice
• Dan-amyloid lesions are associated with dystrophic boutons

cardiovascular system
• at 18 months, mice exhibit age-dependent, vasculature-associated Dan-amyloid deposition in the amygdala, thalamus, brainstem, and cerebellum unlike wild-type mice
• smaller vessels are often obstructed by depositions
• perivascular plaques often surround a significant portion of the vessel surface
• 18 month old mice exhibit cerebral microhemorrhages unlike in wild-type mice
• 18 month old mice exhibit cerebral microhemorrhages unlike in wild-type mice

growth/size
• at 18 months, mice are 20% to 30% lighter than wild-type mice
• coinciding with Dan-amyloid accumulation

skeleton
• at 18 months

hematopoietic system
• in juxtaposition to amyloid depositions

immune system
• in juxtaposition to amyloid depositions

integument
• at 18 months

homeostasis/metabolism
• at 18 months, mice exhibit age-dependent, vasculature-associated Dan-amyloid deposition in the amygdala, thalamus, brainstem, and cerebellum unlike wild-type mice
• smaller vessels are often obstructed by depositions
• perivascular plaques often surround a significant portion of the vessel surface
• perivascular amyloid penetrates into the surrounding parenchyma and is surrounded by microglia and dystrophic neurites

Mouse Models of Human Disease
OMIM IDRef(s)
Cerebral Amyloid Angiopathy, Itm2b-Related, 2 117300 J:159279