A critical concentration of N-terminal pyroglutamylated amyloid beta drives the misfolding of Ab1-42 into more toxic aggregates

Int J Biochem Cell Biol. 2016 Oct:79:261-270. doi: 10.1016/j.biocel.2016.08.037. Epub 2016 Aug 31.

Abstract

A wide consensus based on robust experimental evidence indicates pyroglutamylated amyloid-β isoform (AβpE3-42) as one of the most neurotoxic peptides involved in the onset of Alzheimer's disease. Furthermore, AβpE3-42 co-oligomerized with excess of Aβ1-42, produces oligomers and aggregates that are structurally distinct and far more cytotoxic than those made from Aβ1-42 alone. Here, we investigate quantitatively the influence of AβpE3-42 on biophysical properties and biological activity of Aβ1-42. We tested different ratios of AβpE3-42/Aβ1-42 mixtures finding a correlation between the biological activity and the structural conformation and morphology of the analyzed mixtures. We find that a mixture containing 5% AβpE3-42, induces the highest disruption of intracellular calcium homeostasis and the highest neuronal toxicity. These data correlate to an high content of relaxed antiparallel β-sheet structure and the coexistence of a population of big spheroidal aggregates together with short fibrils. Our experiments provide also evidence that AβpE3-42 causes template-induced misfolding of Aβ1-42 at ratios below 33%. This means that there exists a critical concentration required to have seeding on Aβ1-42 aggregation, above this threshold, the seed effect is not possible anymore and AβpE3-42 controls the total aggregation kinetics.

Keywords: Alzheimer’s disease; Calcium homeostasis; Conformational structure; Morphology; Toxicity; β amyloids.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloid beta-Peptides / chemistry*
  • Amyloid beta-Peptides / toxicity*
  • Animals
  • Calcium / metabolism
  • Cell Survival / drug effects
  • Intracellular Space / drug effects
  • Intracellular Space / metabolism
  • Peptide Fragments / chemistry*
  • Peptide Fragments / toxicity*
  • Protein Aggregates*
  • Protein Conformation, beta-Strand
  • Protein Folding*
  • Pyrrolidonecarboxylic Acid / chemistry*
  • Rats
  • Rats, Sprague-Dawley

Substances

  • Amyloid beta-Peptides
  • Peptide Fragments
  • Protein Aggregates
  • amyloid beta-protein (1-42)
  • Calcium
  • Pyrrolidonecarboxylic Acid