Function of PrP(C) as a copper-binding protein at the synapse

Arch Virol Suppl. 2000:(16):239-49. doi: 10.1007/978-3-7091-6308-5_23.

Abstract

The prion protein (PrP(C)) shows cooperative copper binding of the N-terminal octarepeat (PHGGGWGO) x4. In brain homogenates, PrP(C) is found in highest concentration in synaptosomal fractions. Mice devoid of PrP(C) (Prnp0/0 mice) show synaptosomal copper concentrations diminished by 50% as compared to normal mice. PrP(C) in the synaptic cleft may serve as a copper buffer. Alternatively it may play a role in the re-uptake of copper into the presynapse or may be of structural importance for the N-terminus and thus may influence binding of PrP(C) to other proteins.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Brain / metabolism*
  • Carrier Proteins / metabolism*
  • Copper / metabolism
  • Mice
  • Mice, Knockout
  • Mice, Transgenic
  • PrPC Proteins / genetics
  • PrPC Proteins / metabolism*
  • Synapses / metabolism*

Substances

  • Carrier Proteins
  • PrPC Proteins
  • copper-binding protein
  • Copper