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PMID: 11832240 Published · ppublish English Journal Article Research Support, U.S. Gov't, P.H.S. Review

The utility of prions.

Developmental cell ·Vol. 2 ·No. 2 ·2002-02-00 ·Pages 143-51

Osherovich LZ, Weissman JS

Abstract

Infectious, self-propagating protein aggregates (prions) as well as structurally related amyloid fibrils have traditionally been associated with neurodegenerative diseases in mammals. However, recent work in fungi indicates that prions are not simply aberrations of protein folding, but are in fact widespread, conserved, and in certain cases, apparently beneficial. Analysis of prion behavior in yeast has led to insights into the mechanisms of prion appearance and propagation as well as the effect of prions on cellular physiology and perhaps evolution. The prion-forming proteins of Saccharomyces cerevisiae are members of a larger class of Gln/Asn-rich proteins that is abundantly represented in the genomes of higher eukaryotes, raising the prospect of genetically programmed prion-like behavior in other organisms.

MeSH Terms
Conserved Sequence Evolution, Molecular Humans Models, Biological Molecular Chaperones/metabolism Phenotype Prions/chemistry,genetics,metabolism Protein Binding Protein Structure, Quaternary Saccharomyces cerevisiae/genetics,metabolism Saccharomyces cerevisiae Proteins/chemistry,genetics,metabolism
Chemicals
Molecular Chaperones Prions Saccharomyces cerevisiae Proteins
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Osherovich Lev Z
Howard Hughes Medical Institute, Department of Cellular, University of California, San Francisco 94143, USA. lxoshe@itsa.ucsf.edu
Weissman Jonathan S
Article Info
Journal
Developmental cell
Abbr.
Dev Cell
ISSN
1534-5807
Published
2002-02-00
Pages
143-51
Language
English
Region
United States
NLM ID
101120028
Subset
IM
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