PMID: 9657156Jul 10, 1998Paper

A critical role for amino-terminal glutamine/asparagine repeats in the formation and propagation of a yeast prion

Cell
A H DePaceJonathan S Weissman

Abstract

The yeast [PSI+] factor propagates by a prion-like mechanism involving self-replicating Sup35p amyloids. We identified multiple Sup35p mutants that either are poorly recruited into, or cause curing of, wildtype amyloids in vivo. In vitro, these mutants showed markedly decreased rates of amyloid formation, strongly supporting the protein-only prion hypothesis. Kinetic analysis suggests that the prion state replicates by accelerating slow conformational changes rather than by providing stable nuclei. Strikingly, our mutations map exclusively within a short glutamine/asparagine-rich region of Sup35p, and all but one occur at polar residues. Even after replacement of this region with polyglutamine, Sup35p retains its ability to form amyloids. These and other considerations suggest similarities between the prion-like propagation of [PSI+] and polyglutamine-mediated pathogenesis of several neurodegenerative diseases.

References

Jan 2, 1992·Gene·T W ChristiansonP Hieter
Jan 1, 1991·Methods in Enzymology·F Sherman
Aug 1, 1989·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·W E KlunkD J Abraham
Sep 1, 1988·Yeast·B S CoxC S McLaughlin
Jul 3, 1995·Proceedings of the National Academy of Sciences of the United States of America·K StottM Perutz
Dec 1, 1995·Yeast·R B WicknerH K Edskes
Feb 1, 1997·Chemistry & Biology·J D HarperP T Lansbury
Jun 24, 1997·Proceedings of the National Academy of Sciences of the United States of America·C Y KingK Wüthrich
Jul 18, 1997·Science·S V PaushkinM D Ter-Avanesyan
Sep 18, 1997·Proceedings of the National Academy of Sciences of the United States of America·K KanekoS B Prusiner
Jan 1, 1997·Advances in Protein Chemistry·R Jaenicke, R Seckler
Jan 1, 1997·Advances in Protein Chemistry·M Sunde, C Blake
Jan 1, 1997·Advances in Protein Chemistry·J W KellyS A Peterson
Jan 1, 1997·Advances in Protein Chemistry·R Wetzel
Nov 14, 1997·Journal of Molecular Biology·M SundeC C Blake
Mar 11, 1998·Biochemistry·N D Lazo, D T Downing
May 9, 1998·Proceedings of the National Academy of Sciences of the United States of America·Y LiuD Eisenberg
May 20, 1998·Cell·S B PrusinerF E Cohen
Feb 1, 1997·Trends in Cell Biology·B Caughey, B Chesebro

❮ Previous
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Citations

Apr 7, 2007·Current Genetics·Yakov A VitrenkoSusan W Liebman
Jul 30, 2010·Current Genetics·Alsu F SaifitdinovaAlexey P Galkin
May 12, 2004·Biochimica Et Biophysica Acta·Vladimir N Uversky, Anthony L Fink
Jan 11, 2001·Biophysical Chemistry·J Masel, V A Jansen
Oct 6, 1999·Current Opinion in Neurobiology·J P Brockes
Feb 17, 2000·Trends in Cell Biology·T R Serio, S L Lindquist
Jan 19, 2000·Trends in Microbiology·D C Masison
Apr 5, 2002·Microbes and Infection·Luc Bousset, Ronald Melki
Feb 8, 2002·Developmental Cell·Lev Z Osherovich, Jonathan S Weissman
Sep 2, 2011·Biomacromolecules·Devin M RidgleyJustin R Barone
Dec 20, 2011·Biomacromolecules·Raimon SabatéSalvador Ventura
Nov 3, 2012·The Journal of Physical Chemistry. B·Janarthanan KrishnamoorthyAyyalusamy Ramamoorthy
Jun 10, 2005·Nature·Rajaraman Krishnan, Susan L Lindquist
Jun 10, 2005·Nature·Rebecca NelsonDavid Eisenberg
Dec 31, 2005·Nature Cell Biology·Eric D RossReed B Wickner
Nov 18, 2010·Nature Reviews. Molecular Cell Biology·Mick F Tuite, Tricia R Serio
Jul 17, 2007·Nature Reviews. Microbiology·Reed B WicknerToru Nakayashiki
Jun 6, 2009·Nature Structural & Molecular Biology·Peter M Tessier, Susan Lindquist
Mar 23, 2011·Nature Structural & Molecular Biology·Susanne DiSalvoTricia R Serio
Feb 26, 2013·Nature Structural & Molecular Biology·Bindu L RaveendraAnn E McDermott
Oct 31, 2001·Genes to Cells : Devoted to Molecular & Cellular Mechanisms·Y KimuraT Fujita
Feb 26, 2000·Molecular Microbiology·Y O ChernoffS M Belenkiy
Mar 12, 2002·Molecular Microbiology·V Coustou-LinaresS J Saupe
Feb 28, 2001·Proceedings of the National Academy of Sciences of the United States of America·M BalbirnieD S Eisenberg
Jan 10, 2009·Proceedings of the National Academy of Sciences of the United States of America·Ashley A NenningerScott J Hultgren
Mar 23, 2010·Proceedings of the National Academy of Sciences of the United States of America·Christian AderMarc Baldus
May 21, 2010·Proceedings of the National Academy of Sciences of the United States of America·Sean J GarrityAnn Hochschild
Jun 2, 2011·Proceedings of the National Academy of Sciences of the United States of America·David S CapstickMarie A Elliot
May 24, 2000·Proceedings of the National Academy of Sciences of the United States of America·H K Edskes, R B Wickner
Jun 23, 1999·Proceedings of the National Academy of Sciences of the United States of America·M V KarpujL Steinman
Jan 5, 2000·Proceedings of the National Academy of Sciences of the United States of America·S S EaglestoneM F Tuite
Oct 26, 2000·Proceedings of the National Academy of Sciences of the United States of America·M D Michelitsch, J S Weissman
Aug 15, 2012·The Journal of Biological Chemistry·Yizhou ZhouMatthew R Chapman
May 30, 2008·The Journal of Biological Chemistry·Xuan WangMatthew R Chapman
Dec 15, 2010·Journal of Biomolecular Structure & Dynamics·Youtao SongXing Chen
Dec 17, 2008·Molecular Biology of the Cell·John A PezzaTricia R Serio
Feb 20, 2009·Molecular Biology of the Cell·Lisa A StrawnHeather L True
Mar 12, 2010·Molecular Biology of the Cell·Vidhu MathurSusan W Liebman

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