Related Experiment Video
Updated: Jul 13, 2026
09:22
Monitoring Immune Cells Trafficking Fluorescent Prion Rods Hours after Intraperitoneal Infection
Published on: November 19, 2010
Mice devoid of PrP are resistant to scrapie
Cell
|July 2, 1993
Summary
Mice lacking the normal prion protein (PrPC) resisted scrapie infection, demonstrating PrPC is essential for susceptibility. Introducing foreign prion genes also altered disease resistance, highlighting prion homology
Area of Science:
- Neuroscience
- Molecular Biology
- Infectious Diseases
Background:
- Scrapie is a fatal neurodegenerative disease caused by prions.
- Prions are infectious proteins, specifically PrPSc, a misfolded form of the normal cellular prion protein, PrPC.
- The role of PrPC in scrapie pathogenesis is critical.
Purpose of the Study:
- To investigate the necessity of the normal prion protein (PrPC) for scrapie susceptibility.
- To determine the effect of PrPC gene knockout on disease development.
- To explore the influence of prion homology on disease transmission.
Main Methods:
- Generation of PrPC-deficient (Prn-p0/0) and heterozygous (Prn-p0/+) mice.
- Inoculation of these mice with mouse-adapted scrapie prions.
- Introduction of Syrian hamster PrP transgenes into Prn-p0/0 mice.
- Assessment of scrapie symptom development and survival rates.
Main Results:
- PrPC-deficient mice remained asymptomatic for over 13 months post-inoculation, unlike wild-type controls that died within 6 months.
- Heterozygous Prn-p0/+ mice exhibited enhanced resistance to scrapie.
- Prn-p0/0 mice expressing Syrian hamster PrP became susceptible to hamster prions but not mouse prions.
Conclusions:
- The normal cellular prion protein (PrPC) is required for typical susceptibility to prion diseases like scrapie.
- Lack of homology between the infectious prion and the host PrP gene significantly retards disease progression.
- These findings underscore the critical role of PrPC in prion replication and disease.
