title: Yeast prions: evolution of the prion concept.
abstract: Prions (infectious proteins) analogous to the scrapie agent have been identified in Saccharomyces cerevisiae and Podospora anserina based on their special genetic characteristics. Each is a protein acting as a gene, much like nucleic acids have been shown to act as enzymes. The [URE3], [PSI(+)], [PIN(+)] and [Het-s] prions are self-propagating amyloids of Ure2p, Sup35p, Rnq1p and the HET-s protein, respectively. The [beta] and [C] prions are enzymes whose precursor activation requires their own active form. [URE3] and [PSI(+)] are clearly diseases, while [Het-s] and [beta] carry out normal cell functions. Surprisingly, the prion domains of Ure2p and Sup35p can be randomized without loss of ability to become a prion. Thus amino acid content and not sequence determine these prions. Shuffleability also suggests amyloids with a parallel in-register beta-sheet structure.
authors.affiliation: Laboratory of Biochemistry and Genetics, The National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892-0830, USA. wickner@helix.nih.gov
authors.forename: Reed B
authors.initials: RB
authors.lastname: Wickner
authors.forename: Herman K
authors.initials: HK
authors.lastname: Edskes
authors.forename: Frank
authors.initials: F
authors.lastname: Shewmaker
authors.forename: Toru
authors.initials: T
authors.lastname: Nakayashiki
authors.forename: Abbi
authors.initials: A
authors.lastname: Engel
authors.forename: Linsay
authors.initials: L
authors.lastname: McCann
authors.forename: Dmitry
authors.initials: D
authors.lastname: Kryndushkin
mesh_terms: D000682:Amyloid / Q000235:genetics / Q000378:metabolism*; D048468:Podospora / Q000235:genetics / Q000378:metabolism*; D011328:Prions / Q000235:genetics / Q000378:metabolism*; D017433:Protein Structure, Secondary; D017434:Protein Structure, Tertiary; D012441:Saccharomyces cerevisiae / Q000235:genetics / Q000378:metabolism*; D029701:Saccharomyces cerevisiae Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: D000682:Amyloid; D011328:Prions; D029701:Saccharomyces cerevisiae Proteins