Prions in Humans and Animals

This comprehensive work, aimed at both students and researchers alike, systematically covers all aspects of prion diseases (transmissible spongiform encephalopathies), from their history, microbiology and pathology to their transmissibility and prevention. The book describes diseases such as Creutzfeldt-Jakob disease, kuru, mad cow disease (BSE), chronic wasting disease and scrapie, highlighting their biochemical, molecular biological, genetic, and clinical aspects. A detailed presentation of the impact of prion diseases in fields such as pharmaceutics, blood products, disinfection, surgical i.

50. Evidence for a Link between Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy.

Frontmatter; Table of Contents; Abbreviations; 1. Historical Introduction; 2. History of Kuru Research; 3. History of Prion Research; 4. The Physical Nature of the Prion; 5. Folding of the Recombinant Prion Protein; 6. Structural Studies of Prion Proteins; 7. Function of Cellular Prion Protein (PrPC) in Copper Homeostasis and Redox Signaling at the Synapse; 8. The Scarpie Isoform of the Prion Protein PrPSe Compared to the Celluar Isoform PrPC; 9. The Phylogeny of Mammalian and Nonmammalian Prion Proteins; 10. Knockouts and Transgenic Mice in Prion Research.

11. Transplantation as a Tool in Prion Research12. Prion Strains; 13. Portrait of Kuru; 14. Portrait of Creutzfeldt-Jakob Disease; 15. Portrait of Variant Creutzfeldt-Jakob Disease; 16. Portrait of Gerstmann-Sträussler-Scheinker Disease; 17. Portrait of Fatal Familial Insomnia and Sporadic Fatal Insomnia; 18. Portrait of Scrapie in Sheep and Goat; 19. Portrait of Bovine Spongiform Encephalopathy in Cattle and Other Ungulates; 20. Portrait of Prion Diseases in Zoo Animals; 21. Portrait of Chronic Wasting Disease in Deer Species; 22. Portrait of Transmissible Mink Encephalopathy.

23. Portrait of Transmissible Feline Spongiform …