1. Prions and Protein Misfolding BICH 107 GENE 105

2. Kuru Discovered in Papua New Guinea in early 1900's "Trembling with fear" Characterized by headaches, joint pain, shaking of limbs, progressive dementia Always fatal

3. TSE Transmissible Spongiform Encephalopathy Kuru (human) Creutzfeldt-Jacob Disease (human) Bovine Spongiform Encephalopathy (cow) Scrapie (sheep) Chronic Wasting Disease (elk and deer)

4. Prions Rich in protein No detectable nucleic acid Resistant to treatments that destroy nucleic acids Stanley Prusiner - Nobel Prize in Physiology or Medicine (1997)

5. DNA RNA Protein Information Flow in Cells TranscriptionTranslation

6. Protein Primary Structure

7. H-Bonds in DNA

8.  -Helix Secondary Structure

9.  -sheet Secondary Structure

10. Two Types of  -Sheet

11. Triose-Phosphate Isomerase

12. Triose-Phosphate Isomerase 2

13. Solution Structure of PrP c Copyright © 2000 by the National Academy of Sciences Zahn, Ralph et al. (2000) Proc. Natl. Acad. Sci. USA 97, 145-150

14. Solution Structure of A 

15. Structure of A  fibril Copyright ©2002 by the National Academy of Sciences Petkova, Aneta T. et al. (2002) Proc. Natl. Acad. Sci. USA 99, 16742-16747