Protein misfolding and prion diseases

J Mol Biol. 1999 Oct 22;293(2):313-20. doi: 10.1006/jmbi.1999.2990.

Abstract

The prion diseases provide an intriguing connection between protein folding and neurodegenerative disease. In this review, I explore that importance of protein folding and misfolding in the prion diseases. Thermodynamic and kinetic models are examined in an effort to understand infectious, inherited and sporadic forms of these diseases. These concepts can be generalized to gain insight into other disorders of protein aggregation and deposition such as Alzheimer's disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Animals
  • Humans
  • Kinetics
  • Prion Diseases / genetics
  • Prion Diseases / metabolism*
  • Prions / chemistry*
  • Prions / genetics
  • Prions / metabolism*
  • Protein Folding*
  • Protein Structure, Secondary
  • Thermodynamics

Substances

  • Prions