Prions and Protein Misfolding BICH 107 GENE 105. Kuru Discovered in Papua New Guinea in early 1900's...
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Transcript of Prions and Protein Misfolding BICH 107 GENE 105. Kuru Discovered in Papua New Guinea in early 1900's...
Prions and Protein Misfolding
BICH 107
GENE 105
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
TSE
Transmissible Spongiform Encephalopathy
• Kuru (human)• Creutzfeldt-Jacob Disease (human)• Bovine Spongiform Encephalopathy (cow)• Scrapie (sheep)• Chronic Wasting Disease (elk and deer)
Prions
• Rich in protein• No detectable nucleic acid• Resistant to treatments that destroy nucleic acids• Stanley Prusiner - Nobel Prize in Physiology or
Medicine (1997)
DNA RNA Protein
Information Flow in Cells
Transcription Translation
Protein Primary Structure
H-Bonds in DNA
-Helix Secondary Structure
-sheet Secondary Structure
Two Types of -Sheet
Triose-Phosphate Isomerase
Triose-Phosphate Isomerase 2
Solution Structure of PrPc
Zahn, Ralph et al. (2000) Proc. Natl. Acad. Sci. USA 97, 145-150
Solution Structure of A
Structure of A fibril
Petkova, Aneta T. et al. (2002) Proc. Natl. Acad. Sci. USA 99, 16742-16747