Study of Prion Disease Resistance in Mice with Human Prion Protein Variation
Author Information
Author(s): James F. Striebel, Brent Race, Kimberly D. Meade-White, Rachel LaCasse, Bruce Chesebro
Primary Institution: Laboratory of Persistent Viral Diseases and Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, Hamilton, Montana, United States of America
Hypothesis
Does the N171S polymorphism in human prion protein affect susceptibility to prion diseases in a mouse model?
Conclusion
Transgenic mice expressing the N171S variant of prion protein showed resistance to certain scrapie strains, indicating strain-specific interactions.
Supporting Evidence
- Transgenic mice expressing the N171S variant were resistant to RML and 79A scrapie strains.
- Resistance was not observed with ME7 and 22L scrapie strains.
- Clinical disease in transgenic mice occurred only after 439 days post-inoculation with RML and 79A strains.
Takeaway
Scientists created special mice to see if a human gene change makes them resistant to a brain disease. Some mice were resistant to certain types of the disease, but not all.
Methodology
Transgenic mice expressing the N171S variant of prion protein were tested with four different strains of scrapie to assess susceptibility.
Limitations
The low incidence of the N171S polymorphism in humans limits the ability to study its effects directly in human populations.
Participant Demographics
Transgenic mice expressing the human prion protein variant.
Statistical Information
P-Value
p<0.001
Statistical Significance
p<0.001
Digital Object Identifier (DOI)
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