prions and protein misfolding bich 107 gene 105. kuru discovered in papua new guinea in early...
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Prions and Protein Misfolding
BICH 107
GENE 105
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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
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TSE
Transmissible Spongiform Encephalopathy
• Kuru (human)• Creutzfeldt-Jacob Disease (human)• Bovine Spongiform Encephalopathy (cow)• Scrapie (sheep)• Chronic Wasting Disease (elk and deer)
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Prions
• Rich in protein• No detectable nucleic acid• Resistant to treatments that destroy nucleic acids• Stanley Prusiner - Nobel Prize in Physiology or
Medicine (1997)
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DNA RNA Protein
Information Flow in Cells
Transcription Translation
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Protein Primary Structure
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H-Bonds in DNA
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-Helix Secondary Structure
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-sheet Secondary Structure
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Two Types of -Sheet
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Triose-Phosphate Isomerase
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Triose-Phosphate Isomerase 2
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Solution Structure of PrPc
Zahn, Ralph et al. (2000) Proc. Natl. Acad. Sci. USA 97, 145-150
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Solution Structure of A
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Structure of A fibril
Petkova, Aneta T. et al. (2002) Proc. Natl. Acad. Sci. USA 99, 16742-16747