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1. Introduction
2. Why study prions and prion diseases?
3. Animal prion diseases
4. Human prion diseases
5. Acquired human prion diseases
6. Characteristics of the prion diseases
7. Prions are unique pathogens
8. Convergence of evidence for the prion hypothesis
9. The prion (protein-only) hypothesis
10. Infectious properties of prions - strains
11. Prion strains
12. PrP conformation and prion strains
13. Infectious properties of prions - species barriers
14. Prion species barriers
15. Transgenic modeling of prion transmission barriers
16. Abrogating the species barrier to human prions
17. Chronic wasting disease in North American cervids
18. Development of transgenic mice for studying CWD
19. Mice have a species barrier to CWD prions
20. Transgenic mouse models of CWD
21. Eliminating the barrier to CWD prion transmission
22. Abrogating the species barrier to cwd prions
23. Properties of Tg(CerPrP)1536+/- mice
24. Assessing tissue distribution of CWD prions
25. Bioassay of CWD prions in skeletal muscle
26. Prions in skeletal muscles of deer with CWD
27. Agent distribution in terminally BSE-diseased cow
28. Studies results
29. Cervid PRNP polymorphisms and pathogenesis
30. Codon 129 genotypes in human prion diseases
31. Tg mice expressing L132 CerPrP polymorphisms
32. CWD transmission and matching amino acids
33. CerPrPSc accumulation in Tg(CerPrP) mice
34. CWD-resistant Tg(CerPrP-L132) mice
35. Immunoblotting conformation
36. Conformational stability assay for strain properties
37. Histoblotting for PrP depositions
38. Summary
39. Acknowledgements

Topics Covered

• Prions represent a new biological paradigm of protein-mediated information transfer
• Prions are the cause of fatal, transmissible neurodegenerative diseases, often referred to as transmissible spongiform encephalopathies (TSEs)
• Many unresolved issues remain including the exact molecular nature of the prion, the detailed mechanism of prion propagation, and the mechanism by which prion diseases can be both genetic and infectious
• The factors governing the transmission of prions between species remain unclear, in particular the means by which prion strains and PrP primary structure interact to affect inter-species prion transmission
• Dramatic advances in our understanding of prions have occurred because of their transmissibility to experimental animals and the development of transgenic (Tg) mouse models has done much to further our understanding about various aspects of prion biology

Links

Series:

• Prions and Amyloids

Categories:

• Biochemistry
• Clinical Practice
• Methods
• Neuroscience

Therapeutic Areas:

• Neurology

Talk Citation

Publication History

• Published on September 4, 2008

Financial Disclosures

• Prof. Glenn Telling has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.