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Nascent β Structure in the Elongated Hydrophobic Region of a Gerstmann-Sträussler-Scheinker PrP Allele

Ze-Lin Fu1, Peter C Holmes2, David Westaway1

  • 1Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada T6G 2M8; Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada T6G 2H7.

Journal of Molecular Biology
|April 30, 2019
PubMed

Insights

Gerstmann-Sträussler-Scheinker syndrome, a prion disease, involves an eight-amino-acid insertion in the prion protein (PrPC). This insertion may promote early disease-related structural changes, offering insights into neurodegenerative mechanisms.

Area of Science:

  • Neuroscience
  • Structural Biology
  • Biochemistry

Background:

  • Prion diseases are fatal neurodegenerative disorders.
  • Caused by misfolding of cellular prion protein (PrPC).
  • Gerstmann-Sträussler-Scheinker syndrome (GSS) is an inherited prion disease linked to specific PrPC mutations.

Purpose of the Study:

  • Characterize the structure, motion, and dynamics of the HRdup allele in GSS.
  • Investigate the impact of the LGGLGGYV insertion on PrPC structure.
  • Explore factors influencing conformational dynamics in PrPC.

Main Methods:

  • High-resolution Nuclear Magnetic Resonance (NMR) spectroscopy.
  • Structural and dynamic analysis of HRdup and control PrP alleles.
  • Investigation using both human and mouse PrPC sequences.

Main Results:

  • HRdup PrP core structure is similar to canonical PrPC.
  • A nascent β-structure was identified in the elongated hydrophobic region of HRdup.
  • Methionine/valine polymorphism and high protein concentration affect conformational dynamics.

Conclusions:

  • The HRdup insertion may induce early β-structure formation in the hydrophobic region.
  • This structural change could facilitate extended β-sheet formation, contributing to disease pathogenesis.
  • Understanding these structural events is crucial for GSS and other prion disease research.

Keywords:
GSS diseaseNMR structuremotion dynamicsoligomerizationprion protein

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