Solid-state NMR studies of the secondary structure of a mutant prion protein fragment of 55 residues that induces neurodegeneration

Proc Natl Acad Sci U S A. 2001 Sep 25;98(20):11686-90. doi: 10.1073/pnas.201404298. Epub 2001 Sep 18.

Abstract

The secondary structure of a 55-residue fragment of the mouse prion protein, MoPrP(89-143), was studied in randomly aggregated (dried from water) and fibrillar (precipitated from water/acetonitrile) forms by (13)C solid-state NMR. Recent studies have shown that the fibrillar form of the P101L mutant of MoPrP(89-143) is capable of inducing prion disease in transgenic mice, whereas unaggregated or randomly aggregated samples do not provoke disease. Through analysis of (13)C chemical shifts, we have determined that both wild-type and mutant sequence MoPrP(89-143) form a mixture of beta-sheet and alpha-helical conformations in the randomly aggregated state although the beta-sheet content in MoPrP(89-143, P101L) is significantly higher than in the wild-type peptide. In a fibrillar state, MoPrP(89-143, P101L) is completely converted into beta-sheet, suggesting that the formation of a specific beta-sheet structure may be required for the peptide to induce disease. Studies of an analogous peptide from Syrian hamster PrP verify that sequence alterations in residues 101-117 affect the conformation of aggregated forms of the peptides.

Publication types

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

MeSH terms

  • Amino Acid Sequence
  • Amino Acid Substitution
  • Animals
  • Carbon Isotopes
  • Isotope Labeling / methods
  • Mice
  • Mice, Transgenic
  • Molecular Sequence Data
  • Nuclear Magnetic Resonance, Biomolecular / methods
  • Peptide Fragments / chemistry*
  • PrPC Proteins / chemistry*
  • Prion Diseases
  • Prions / chemistry*
  • Protein Conformation
  • Protein Structure, Secondary
  • Sequence Alignment

Substances

  • Carbon Isotopes
  • Peptide Fragments
  • PrPC Proteins
  • Prions