NMR-detected hydrogen exchange and molecular dynamics simulations provide structural insight into fibril formation of prion protein fragment 106-126

Proc Natl Acad Sci U S A. 2003 Dec 9;100(25):14790-5. doi: 10.1073/pnas.2433563100. Epub 2003 Dec 1.

Abstract

PrP106-126, a peptide corresponding to residues 107-127 of the human prion protein, induces neuronal cell death by apoptosis and causes proliferation and hypertrophy of glia, reproducing the main neuropathological features of prion-related transmissible spongiform encephalopathies, such as bovine spongiform encephalopathy and Creutzfeldt-Jakob disease. Although PrP106-126 has been shown to form amyloid-like fibrils in vitro, their structural properties have not been elucidated. Here, we investigate the conformational characteristics of a fibril-forming fragment of the mouse prion protein, MoPrP106-126, by using electron microscopy, CD spectroscopy, NMR-detected hydrogen-deuterium exchange measurements, and molecular dynamics simulations. The fibrils contain approximately 50% beta-sheet structure, and strong amide exchange protection is limited to the central portion of the peptide spanning the palindromic sequence VAGAAAAGAV. Molecular dynamics simulations indicate that MoPrP106-126 in water assumes a stable structure consisting of two four-stranded parallel beta-sheets that are tightly packed against each other by methyl-methyl interactions. Fibril formation involving polyalanine stacking is consistent with the experimental observations.

Publication types

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

MeSH terms

  • Animals
  • Circular Dichroism
  • Hydrogen / chemistry*
  • Magnetic Resonance Spectroscopy
  • Mice
  • Microscopy, Electron
  • Models, Molecular
  • Neurons / metabolism
  • Peptide Fragments / chemistry*
  • Peptides / chemistry
  • Prions / chemistry*
  • Protein Conformation
  • Protein Structure, Secondary

Substances

  • Peptide Fragments
  • Peptides
  • Prions
  • prion protein (106-126)
  • Hydrogen