Lecture - Prion Diseases

Lecture - Prion Diseases - PRION DISEASES MAD COW (BSE)...

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Click to edit Master subtitle style P RION D ISEASES M AD C OW (BSE) Presented by: R ICHARD M. T OVAR
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Kuru l Fore People Tremors/Dementia 1957-1968 (M1T) Cannibal Rituals TSEs Information
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Overview I. Prion Diseases II. Impact of BSE III. Pathogenicity IV. New Developments V. Conclusions
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I. Prion Diseases l Definition Self-Replicating (Rogue Protein) Cause TSE’s?
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Prion (Continued)
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Prion (Continued) l BSE Cattle Infected Feed (Scrapie) D3-6Mo
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Prion (Continued) l CJD Older Adults (50Y-75Y) Sporadic? l 150/Y:D10Y
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Prion (Continued) l vCJD Young Adults (20Y-40Y) Infected Beef P137 (‘95): D1-3Y
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Prion (Continued) l BSE Signs/Symptoms Nervous/ A Weight/ ƒ Walking
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Prion (Continued) l vCJD Signs/Symptoms Amnesia/Rapid Dementia/Tremors
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Prion (Continued) l Diagnosis Prion Separation Proteinase K PMortemBO Sponge-Like
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Prion (Continued) l Treatment None (Fatal) Clinical Trials Cremation Infected Suspected
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II. Impact of BSE l Time Line 1984 UK BSE1st 1995 UK vCJD 2003 CA BSE1st 2003 US BSE1st 2008 CA BSE15th
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Impact (Continued) l UK/EU ~200T(K6M) Economic Loss €40BUK/€100BEU 300TUK Jobs
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Impact (Continued) l CANADA K1.76M Economic Loss $11M/Day 90T Jobs
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Impact (Continued) l US #1 P/C (100M) 18% Farm Sales Estimated Loss $30BDEO $90BREO
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III. Mechanism l Overview Stanley Prusiner Cellular PrPC Rogue PrPSc
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Mechanism (Continued) l PrPC Glycoprotein Digestible Gene PRNP
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Mechanism (Continued) l PrPSc Rouge Protein Indigestible PrPC& PrPSc
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Mechanism (Continued) l Spread Axons (Retrograde Flow) Blood Lymphocyte Astrocyte
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