Co-fibrillogenesis of Wild-type and D76N β2-Microglobulin: THE CRUCIAL ROLE OF FIBRILLAR SEEDS

J Biol Chem. 2016 Apr 29;291(18):9678-89. doi: 10.1074/jbc.M116.720573. Epub 2016 Feb 26.

Abstract

The amyloidogenic variant of β2-microglobulin, D76N, can readily convert into genuine fibrils under physiological conditions and primes in vitro the fibrillogenesis of the wild-type β2-microglobulin. By Fourier transformed infrared spectroscopy, we have demonstrated that the amyloid transformation of wild-type β2-microglobulin can be induced by the variant only after its complete fibrillar conversion. Our current findings are consistent with preliminary data in which we have shown a seeding effect of fibrils formed from D76N or the natural truncated form of β2-microglobulin lacking the first six N-terminal residues. Interestingly, the hybrid wild-type/variant fibrillar material acquired a thermodynamic stability similar to that of homogenous D76N β2-microglobulin fibrils and significantly higher than the wild-type homogeneous fibrils prepared at neutral pH in the presence of 20% trifluoroethanol. These results suggest that the surface of D76N β2-microglobulin fibrils can favor the transition of the wild-type protein into an amyloid conformation leading to a rapid integration into fibrils. The chaperone crystallin, which is a mild modulator of the lag phase of the variant fibrillogenesis, potently inhibits fibril elongation of the wild-type even once it is absorbed on D76N β2-microglobulin fibrils.

Keywords: Fourier transform IR (FTIR); amyloid; fibril; protein aggregation; protein misfolding; β2-microglobulin.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Substitution
  • Amyloid / chemistry*
  • Amyloid / genetics
  • Amyloid / metabolism
  • Crystallins / chemistry
  • Crystallins / genetics
  • Crystallins / metabolism
  • Humans
  • Hydrogen-Ion Concentration
  • Molecular Chaperones / chemistry
  • Molecular Chaperones / genetics
  • Molecular Chaperones / metabolism
  • Mutation, Missense*
  • Protein Aggregation, Pathological*
  • beta 2-Microglobulin / chemistry*
  • beta 2-Microglobulin / genetics
  • beta 2-Microglobulin / metabolism

Substances

  • Amyloid
  • Crystallins
  • Molecular Chaperones
  • beta 2-Microglobulin