Structural and hydration properties of the partially unfolded states of the prion protein

Biophys J. 2007 Aug 15;93(4):1284-92. doi: 10.1529/biophysj.107.108613. Epub 2007 May 4.

Abstract

Misfolding and aggregation of the prion protein (PrP) is responsible for the development of transmissible spongiform encephalopathies (TSE). To gain insights into possible aggregation-prone intermediate states, we construct the free energy surface of the C-terminal globular domain of the PrP from enhanced sampling of replica exchange molecular dynamics. This cellular domain is characterized by three helices H1-H3 and a small beta-sheet. In agreement with experimental studies, the partially unfolded states display a stable core built from the central portions of helices H2 and H3 and a high mobility of helix H1 from the core. Among all identified conformational basins, a marginally populated state appears to be a very good candidate for aggregation. This intermediate is stabilized by four TSE-sensitive key interactions, displays a longer helix H1 with both a dry and solvated surface, and is featured by a significant detachment of helix H1 from the PrP-core.

MeSH terms

  • Models, Molecular*
  • PrPC Proteins / chemistry*
  • Protein Folding*
  • Protein Structure, Secondary
  • Water / chemistry*

Substances

  • PrPC Proteins
  • Water