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Candidate Prion Host Protein Affected Effect of the Prion Protein Alteration Scrapie, CJD, Mad Cow Dis., etc. Mammals PrP Ataxia, dementia, death Protease-resistance, aggregation, amyloid formation, increased beta sheet [URE3] Yeast (S. cerevisiae) Ure2p Derepressed nitrogen regulation Protease-resistance, aggregation, in vitro amyloid formation [PSI] Yeast (S. cerevisiae) Sup35p Readthrough of translation termination codons Protease-resistance, aggregation, in vitro filament formation [Het-s] Podospora (a filamentous fungus) Het-s protein Heterokaryon incompatibility not known
Page last updated: December 18, 2008