Signature of an aggregation-prone conformation of tau

Sci Rep. 2017 Mar 17:7:44739. doi: 10.1038/srep44739.

Abstract

The self-assembly of the microtubule associated tau protein into fibrillar cell inclusions is linked to a number of devastating neurodegenerative disorders collectively known as tauopathies. The mechanism by which tau self-assembles into pathological entities is a matter of much debate, largely due to the lack of direct experimental insights into the earliest stages of aggregation. We present pulsed double electron-electron resonance measurements of two key fibril-forming regions of tau, PHF6 and PHF6*, in transient as aggregation happens. By monitoring the end-to-end distance distribution of these segments as a function of aggregation time, we show that the PHF6(*) regions dramatically extend to distances commensurate with extended β-strand structures within the earliest stages of aggregation, well before fibril formation. Combined with simulations, our experiments show that the extended β-strand conformational state of PHF6(*) is readily populated under aggregating conditions, constituting a defining signature of aggregation-prone tau, and as such, a possible target for therapeutic interventions.

Publication types

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

MeSH terms

  • Amino Acid Sequence
  • Electrons
  • Heparin / pharmacology
  • Molecular Dynamics Simulation
  • Mutant Proteins / chemistry
  • Peptides / chemistry
  • Protein Aggregates*
  • Protein Conformation
  • Solutions
  • Time Factors
  • tau Proteins / chemistry*
  • tau Proteins / ultrastructure

Substances

  • Mutant Proteins
  • Peptides
  • Protein Aggregates
  • Solutions
  • tau Proteins
  • Heparin