Prion protein binds copper within the physiological concentration range

J Biol Chem. 2001 May 18;276(20):16711-9. doi: 10.1074/jbc.M006554200. Epub 2001 Feb 27.

Abstract

The prion protein is known to be a copper-binding protein, but affinity and stoichiometry data for the full-length protein at a physiological pH of 7 were lacking. Furthermore, it was unknown whether only the highly flexible N-terminal segment with its octarepeat region is involved in copper binding or whether the structured C-terminal domain is also involved. Therefore we systematically investigated the stoichiometry and affinity of copper binding to full-length prion protein PrP(23-231) and to different N- and C-terminal fragments using electrospray ionization mass spectrometry and fluorescence spectroscopy. Our data indicate that the unstructured N-terminal segment is the cooperative copper-binding domain of the prion protein. The prion protein binds up to five copper(II) ions with half-maximal binding at approximately 2 microm. This argues strongly for a direct role of the prion protein in copper metabolism, since it is almost saturated at about 5 microm, and the exchangeable copper pool concentration in blood is about 8 microm.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Binding Sites
  • Copper / metabolism*
  • Humans
  • Hydrogen-Ion Concentration
  • Kinetics
  • Mice
  • Peptide Fragments / chemical synthesis
  • Peptide Fragments / chemistry
  • Peptide Fragments / metabolism*
  • Prions / chemistry*
  • Prions / metabolism*
  • Protein Conformation
  • Spectrometry, Fluorescence
  • Spectrometry, Mass, Electrospray Ionization

Substances

  • Peptide Fragments
  • Prions
  • Copper