Molecular chaperones antagonize proteotoxicity by differentially modulating protein aggregation pathways

Prion. 2009 Apr-Jun;3(2):51-8. doi: 10.4161/pri.3.2.8587. Epub 2009 Apr 26.

Abstract

The self-association of misfolded or damaged proteins into ordered amyloid-like aggregates characterizes numerous neurodegenerative disorders. Insoluble amyloid plaques are diagnostic of many disease states. Yet soluble, oligomeric intermediates in the aggregation pathway appear to represent the toxic culprit. Molecular chaperones regulate the fate of misfolded proteins and thereby influence their aggregation state. Chaperones conventionally antagonize aggregation of misfolded, disease proteins and assist in refolding or degradation pathways. Recent work suggests that chaperones may also suppress neurotoxicity by converting toxic, soluble oligomers into benign aggregates. Chaperones can therefore suppress or promote aggregation of disease proteins to ameliorate the proteotoxic accumulation of soluble, assembly intermediates.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Amyloid / metabolism
  • Animals
  • Humans
  • Models, Biological
  • Molecular Chaperones / metabolism
  • Molecular Chaperones / physiology*
  • Neurodegenerative Diseases / metabolism
  • Protein Folding
  • Proteins / metabolism*

Substances

  • Amyloid
  • Molecular Chaperones
  • Proteins