η-Secretase processing of APP inhibits neuronal activity in the hippocampus

Nature. 2015 Oct 15;526(7573):443-7. doi: 10.1038/nature14864. Epub 2015 Aug 31.

Abstract

Alzheimer disease (AD) is characterized by the accumulation of amyloid plaques, which are predominantly composed of amyloid-β peptide. Two principal physiological pathways either prevent or promote amyloid-β generation from its precursor, β-amyloid precursor protein (APP), in a competitive manner. Although APP processing has been studied in great detail, unknown proteolytic events seem to hinder stoichiometric analyses of APP metabolism in vivo. Here we describe a new physiological APP processing pathway, which generates proteolytic fragments capable of inhibiting neuronal activity within the hippocampus. We identify higher molecular mass carboxy-terminal fragments (CTFs) of APP, termed CTF-η, in addition to the long-known CTF-α and CTF-β fragments generated by the α- and β-secretases ADAM10 (a disintegrin and metalloproteinase 10) and BACE1 (β-site APP cleaving enzyme 1), respectively. CTF-η generation is mediated in part by membrane-bound matrix metalloproteinases such as MT5-MMP, referred to as η-secretase activity. η-Secretase cleavage occurs primarily at amino acids 504-505 of APP695, releasing a truncated ectodomain. After shedding of this ectodomain, CTF-η is further processed by ADAM10 and BACE1 to release long and short Aη peptides (termed Aη-α and Aη-β). CTFs produced by η-secretase are enriched in dystrophic neurites in an AD mouse model and in human AD brains. Genetic and pharmacological inhibition of BACE1 activity results in robust accumulation of CTF-η and Aη-α. In mice treated with a potent BACE1 inhibitor, hippocampal long-term potentiation was reduced. Notably, when recombinant or synthetic Aη-α was applied on hippocampal slices ex vivo, long-term potentiation was lowered. Furthermore, in vivo single-cell two-photon calcium imaging showed that hippocampal neuronal activity was attenuated by Aη-α. These findings not only demonstrate a major functionally relevant APP processing pathway, but may also indicate potential translational relevance for therapeutic strategies targeting APP processing.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • ADAM Proteins / metabolism
  • ADAM10 Protein
  • Alzheimer Disease / enzymology
  • Alzheimer Disease / metabolism
  • Amyloid Precursor Protein Secretases / antagonists & inhibitors
  • Amyloid Precursor Protein Secretases / cerebrospinal fluid
  • Amyloid Precursor Protein Secretases / deficiency
  • Amyloid Precursor Protein Secretases / genetics
  • Amyloid Precursor Protein Secretases / metabolism*
  • Amyloid beta-Protein Precursor / cerebrospinal fluid
  • Amyloid beta-Protein Precursor / chemistry
  • Amyloid beta-Protein Precursor / genetics
  • Amyloid beta-Protein Precursor / metabolism*
  • Animals
  • Aspartic Acid Endopeptidases / antagonists & inhibitors
  • Aspartic Acid Endopeptidases / deficiency
  • Aspartic Acid Endopeptidases / genetics
  • Aspartic Acid Endopeptidases / metabolism
  • Calcium Signaling
  • Disease Models, Animal
  • Female
  • Hippocampus / cytology*
  • Hippocampus / enzymology
  • Hippocampus / physiology
  • Humans
  • In Vitro Techniques
  • Long-Term Potentiation
  • Male
  • Matrix Metalloproteinases, Membrane-Associated / deficiency
  • Matrix Metalloproteinases, Membrane-Associated / metabolism*
  • Membrane Proteins / metabolism
  • Mice
  • Molecular Weight
  • Neurites / enzymology
  • Neurites / metabolism
  • Neurons / enzymology
  • Neurons / physiology*
  • Peptide Fragments / chemistry
  • Peptide Fragments / metabolism
  • Plaque, Amyloid
  • Protein Processing, Post-Translational
  • Proteolysis*
  • Single-Cell Analysis

Substances

  • APP protein, human
  • Amyloid beta-Protein Precursor
  • Membrane Proteins
  • Peptide Fragments
  • Amyloid Precursor Protein Secretases
  • Aspartic Acid Endopeptidases
  • BACE1 protein, human
  • Bace1 protein, mouse
  • ADAM Proteins
  • Matrix Metalloproteinases, Membrane-Associated
  • Mmp24 protein, mouse
  • ADAM10 Protein
  • ADAM10 protein, human