Increasing prion propensity by hydrophobic insertion.

Prion formation involves the conversion of proteins from a soluble form into an infectious amyloid form. Most yeast prion proteins contain glutamine/asparagine-rich regions that are responsible for prion aggregation. Prion formation by these domains is driven primarily by amino acid composition, not...

Full description

Saved in:
Bibliographic Details
Main Authors: Aaron C Gonzalez Nelson, Kacy R Paul, Michelina Petri, Noe Flores, Ryan A Rogge, Sean M Cascarina, Eric D Ross
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0089286&type=printable
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850024147238780928
author Aaron C Gonzalez Nelson
Kacy R Paul
Michelina Petri
Noe Flores
Ryan A Rogge
Sean M Cascarina
Eric D Ross
author_facet Aaron C Gonzalez Nelson
Kacy R Paul
Michelina Petri
Noe Flores
Ryan A Rogge
Sean M Cascarina
Eric D Ross
author_sort Aaron C Gonzalez Nelson
collection DOAJ
description Prion formation involves the conversion of proteins from a soluble form into an infectious amyloid form. Most yeast prion proteins contain glutamine/asparagine-rich regions that are responsible for prion aggregation. Prion formation by these domains is driven primarily by amino acid composition, not primary sequence, yet there is a surprising disconnect between the amino acids thought to have the highest aggregation propensity and those that are actually found in yeast prion domains. Specifically, a recent mutagenic screen suggested that both aromatic and non-aromatic hydrophobic residues strongly promote prion formation. However, while aromatic residues are common in yeast prion domains, non-aromatic hydrophobic residues are strongly under-represented. Here, we directly test the effects of hydrophobic and aromatic residues on prion formation. Remarkably, we found that insertion of as few as two hydrophobic residues resulted in a multiple orders-of-magnitude increase in prion formation, and significant acceleration of in vitro amyloid formation. Thus, insertion or deletion of hydrophobic residues provides a simple tool to control the prion activity of a protein. These data, combined with bioinformatics analysis, suggest a limit on the number of strongly prion-promoting residues tolerated in glutamine/asparagine-rich domains. This limit may explain the under-representation of non-aromatic hydrophobic residues in yeast prion domains. Prion activity requires not only that a protein be able to form prion fibers, but also that these fibers be cleaved to generate new independently-segregating aggregates to offset dilution by cell division. Recent studies suggest that aromatic residues, but not non-aromatic hydrophobic residues, support the fiber cleavage step. Therefore, we propose that while both aromatic and non-aromatic hydrophobic residues promote prion formation, aromatic residues are favored in yeast prion domains because they serve a dual function, promoting both prion formation and chaperone-dependent prion propagation.
format Article
id doaj-art-b6a519afec7c42f08aa9f0deaf66ea7e
institution DOAJ
issn 1932-6203
language English
publishDate 2014-01-01
publisher Public Library of Science (PLoS)
record_format Article
series PLoS ONE
spelling doaj-art-b6a519afec7c42f08aa9f0deaf66ea7e2025-08-20T03:01:11ZengPublic Library of Science (PLoS)PLoS ONE1932-62032014-01-0192e8928610.1371/journal.pone.0089286Increasing prion propensity by hydrophobic insertion.Aaron C Gonzalez NelsonKacy R PaulMichelina PetriNoe FloresRyan A RoggeSean M CascarinaEric D RossPrion formation involves the conversion of proteins from a soluble form into an infectious amyloid form. Most yeast prion proteins contain glutamine/asparagine-rich regions that are responsible for prion aggregation. Prion formation by these domains is driven primarily by amino acid composition, not primary sequence, yet there is a surprising disconnect between the amino acids thought to have the highest aggregation propensity and those that are actually found in yeast prion domains. Specifically, a recent mutagenic screen suggested that both aromatic and non-aromatic hydrophobic residues strongly promote prion formation. However, while aromatic residues are common in yeast prion domains, non-aromatic hydrophobic residues are strongly under-represented. Here, we directly test the effects of hydrophobic and aromatic residues on prion formation. Remarkably, we found that insertion of as few as two hydrophobic residues resulted in a multiple orders-of-magnitude increase in prion formation, and significant acceleration of in vitro amyloid formation. Thus, insertion or deletion of hydrophobic residues provides a simple tool to control the prion activity of a protein. These data, combined with bioinformatics analysis, suggest a limit on the number of strongly prion-promoting residues tolerated in glutamine/asparagine-rich domains. This limit may explain the under-representation of non-aromatic hydrophobic residues in yeast prion domains. Prion activity requires not only that a protein be able to form prion fibers, but also that these fibers be cleaved to generate new independently-segregating aggregates to offset dilution by cell division. Recent studies suggest that aromatic residues, but not non-aromatic hydrophobic residues, support the fiber cleavage step. Therefore, we propose that while both aromatic and non-aromatic hydrophobic residues promote prion formation, aromatic residues are favored in yeast prion domains because they serve a dual function, promoting both prion formation and chaperone-dependent prion propagation.https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0089286&type=printable
spellingShingle Aaron C Gonzalez Nelson
Kacy R Paul
Michelina Petri
Noe Flores
Ryan A Rogge
Sean M Cascarina
Eric D Ross
Increasing prion propensity by hydrophobic insertion.
PLoS ONE
title Increasing prion propensity by hydrophobic insertion.
title_full Increasing prion propensity by hydrophobic insertion.
title_fullStr Increasing prion propensity by hydrophobic insertion.
title_full_unstemmed Increasing prion propensity by hydrophobic insertion.
title_short Increasing prion propensity by hydrophobic insertion.
title_sort increasing prion propensity by hydrophobic insertion
url https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0089286&type=printable
work_keys_str_mv AT aaroncgonzaleznelson increasingprionpropensitybyhydrophobicinsertion
AT kacyrpaul increasingprionpropensitybyhydrophobicinsertion
AT michelinapetri increasingprionpropensitybyhydrophobicinsertion
AT noeflores increasingprionpropensitybyhydrophobicinsertion
AT ryanarogge increasingprionpropensitybyhydrophobicinsertion
AT seanmcascarina increasingprionpropensitybyhydrophobicinsertion
AT ericdross increasingprionpropensitybyhydrophobicinsertion