γ-Secretase Modulators and APH1 Isoforms Modulate γ-Secretase Cleavage but Not Position of ε-Cleavage of the Amyloid Precursor Protein (APP)

PLoS One. 2015 Dec 17;10(12):e0144758. doi: 10.1371/journal.pone.0144758. eCollection 2015.

Abstract

The relative increase in Aβ42 peptides from familial Alzheimer disease (FAD) linked APP and PSEN mutations can be related to changes in both ε-cleavage site utilization and subsequent step-wise cleavage. Cleavage at the ε-site releases the amyloid precursor protein (APP) intracellular domain (AICD), and perturbations in the position of ε-cleavage are closely associated with changes in the profile of amyloid β-protein (Aβ) species that are produced and secreted. The mechanisms by which γ-secretase modulators (GSMs) or FAD mutations affect the various γ-secretase cleavages to alter the generation of Aβ peptides have not been fully elucidated. Recent studies suggested that GSMs do not modulate ε-cleavage of APP, but the data were derived principally from recombinant truncated epitope tagged APP substrate. Here, using full length APP from transfected cells, we investigated whether GSMs modify the ε-cleavage of APP under more native conditions. Our results confirmed the previous findings that ε-cleavage is insensitive to GSMs. In addition, fenofibrate, an inverse GSM (iGSM), did not alter the position or kinetics of ε-cleavage position in vitro. APH1A and APH1B, a subunit of the γ-secretase complex, also modulated Aβ42/Aβ40 ratio without any alterations in ε-cleavage, a result in contrast to what has been observed with PS1 and APP FAD mutations. Consequently, GSMs and APH1 appear to modulate γ-secretase activity and Aβ42 generation by altering processivity but not ε-cleavage site utilization.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Amyloid Precursor Protein Secretases / drug effects
  • Amyloid Precursor Protein Secretases / metabolism
  • Amyloid Precursor Protein Secretases / physiology*
  • Amyloid beta-Protein Precursor / genetics
  • Amyloid beta-Protein Precursor / metabolism*
  • Animals
  • CHO Cells
  • Catalytic Domain
  • Cricetulus
  • Endopeptidases
  • Fenofibrate / pharmacology
  • HEK293 Cells
  • Humans
  • Immunoprecipitation
  • Membrane Proteins / genetics
  • Membrane Proteins / physiology*
  • Mutation
  • Peptide Fragments / metabolism
  • Peptide Hydrolases / genetics
  • Peptide Hydrolases / physiology*
  • Protein Isoforms / physiology
  • Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization

Substances

  • Amyloid beta-Protein Precursor
  • Membrane Proteins
  • Peptide Fragments
  • Protein Isoforms
  • amyloid beta-protein precursor C-terminal fragment beta, human
  • APH1A protein, human
  • Amyloid Precursor Protein Secretases
  • Endopeptidases
  • Peptide Hydrolases
  • Fenofibrate

Associated data

  • figshare/10.6084/M9.FIGSHARE.1597667