The amyloid precursor protein has a flexible transmembrane domain and binds cholesterol

Science. 2012 Jun 1;336(6085):1168-71. doi: 10.1126/science.1219988.

Abstract

C99 is the transmembrane carboxyl-terminal domain of the amyloid precursor protein that is cleaved by γ-secretase to release the amyloid-β polypeptides, which are associated with Alzheimer's disease. Nuclear magnetic resonance and electron paramagnetic resonance spectroscopy show that the extracellular amino terminus of C99 includes a surface-embedded "N-helix" followed by a short "N-loop" connecting to the transmembrane domain (TMD). The TMD is a flexibly curved α helix, making it well suited for processive cleavage by γ-secretase. Titration of C99 reveals a binding site for cholesterol, providing mechanistic insight into how cholesterol promotes amyloidogenesis. Membrane-buried GXXXG motifs (G, Gly; X, any amino acid), which have an established role in oligomerization, were also shown to play a key role in cholesterol binding. The structure and cholesterol binding properties of C99 may aid in the design of Alzheimer's therapeutics.

Publication types

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

MeSH terms

  • Amino Acid Motifs
  • Amino Acid Sequence
  • Amyloid beta-Protein Precursor / chemistry*
  • Amyloid beta-Protein Precursor / genetics
  • Amyloid beta-Protein Precursor / metabolism*
  • Binding Sites
  • Cholesterol / metabolism*
  • Electron Spin Resonance Spectroscopy
  • Humans
  • Micelles
  • Molecular Sequence Data
  • Mutation
  • Nuclear Magnetic Resonance, Biomolecular
  • Peptide Fragments / chemistry*
  • Peptide Fragments / genetics
  • Peptide Fragments / metabolism*
  • Protein Binding
  • Protein Structure, Secondary
  • Protein Structure, Tertiary

Substances

  • Amyloid beta-Protein Precursor
  • Micelles
  • Peptide Fragments
  • amyloid beta-protein precursor C-terminal fragment beta, human
  • Cholesterol

Associated data

  • PDB/2LP1