homeostasis/metabolism
• blood calcium level is significantly higher than in wild-type controls
|
• blood alkaline phosphatase level (ALP) levels are ~3 times higher than in wild-type controls, suggesting that the cell surface level of ALP is reduced
|
skeleton
short tibia
(
J:333707
)
|
• females show a significantly shorter tibia length (mm) than wild-type controls
|
|
• 42.9% of males exhibit bowed ribs
|
|
• 42.9% of males exhibit a caved-in ribcage
|
|
• 57.1% of males show abnormally shaped cervical vertebrae
• however, % of males with a deformed ventral tubercle of atlas is equivalent to that in wild-type controls (14.3%)
|
|
• 57.1% of females show a deformed ventral tubercle of atlas relative to 14.3% in wild-type controls
|
|
• males exhibit a significantly smaller bone area (cm2) than wild-type controls
|
• bone mineral content (g) is significantly lower than in wild-type controls
|
behavior/neurological
• in a contextual fear conditioning test, mice show a lower % of freezing time at each time point analyzed, suggesting impaired memory formation
|
• in a sociability test, mice spend more time in front of the cage near an unstimulating novel object than wild-type controls
• however, time spent near a stranger mouse is comparable to that in wild-type controls
|
• in the open field test, the distance traveled is decreased, particularly in the periphery of the field, while the average speed is significantly reduced, indicating reduced locomotion activity
• average home cage activity is significantly lower than that in wild-type controls in the dark, but not in the light, state
• however, the % of time spent in the center region and latency to center entry in the open field are normal, suggesting lack of anxiety-like behaviors
|
immune system
|
• females show a slight increase in CD11b- Ly6C+ NK cells
|
• after intracerebral inoculation with pathogenic PrPSc strains (Fukuoka-1 or Chandler), mice die significantly earlier than similarly inoculated wild-type controls, with an average survival of less than 150 days post inoculation (dpi)
|
• after intracerebral inoculation with Fukuoka-1 (a mouse-adapted Gerstmann-Straussler-Scheinker syndrome prion strain) or Chandler (a mouse-adapted scrapie prion strain), mice exhibit a significantly shorter incubation period but no differences in PrPSc accumulation or levels of gliosis in deceased (endpoint) brains relative to similarly inoculated wild-type controls
• at 50 days dpi with the Fukuoka-1 strain, PrPSc accumulation is robustly increased relative to wild-type controls, indicating accelerated conversion of PrPC to PrPSc
|
limbs/digits/tail
short tibia
(
J:333707
)
|
• females show a significantly shorter tibia length (mm) than wild-type controls
|
growth/size/body
|
• males show a significantly higher body fat mass (g) than wild-type controls
|
cardiovascular system
|
• males exhibit a slight increase in mean R amplitude (mV), suggesting that the left ventricle is enlarged
|
adipose tissue
|
• males show a significantly higher body fat mass (g) than wild-type controls
|
|
• males show a significantly higher body fat percentage (%) than wild-type controls
|
hematopoietic system
|
• females show a slight increase in CD11b- Ly6C+ NK cells
|
mortality/aging
• after intracerebral inoculation with pathogenic PrPSc strains (Fukuoka-1 or Chandler), mice die significantly earlier than similarly inoculated wild-type controls, with an average survival of less than 150 days post inoculation (dpi)
|
nervous system
N |
• brain histology showed no apparent abnormalities in the cortex, hippocampus, thalamus, cerebellum or medulla oblongata; no defects in prepulse inhibition or epileptic symptoms are observed
|