A human prion protein peptide (PrP(59-91)) protects against copper neurotoxicity

Mol Psychiatry. 2003 Oct;8(10):853-62, 835. doi: 10.1038/sj.mp.4001400.

Abstract

Human cellular prion protein (PrP(C)) is involved in several neurodegenerative disorders; however, its normal function is unknown. We report here that a synthetic peptide corresponding to the four-octarepeat sequence of the PrP(C) (PrP(59-91)) protects hippocampal neurons against copper neurotoxic effects in vivo. Using a rat bilateral intrahippocampal injection model, we found that PrP(59-91) protects against copper-induced neurotoxicity, including a recovery in spatial learning performance and a reduced neuronal cell loss and astrogliosis. Previous studies from our laboratory indicated that a tryptophan (Trp) residue plays a key role in the reduction of copper(II) to copper(I); therefore several PrP(59-91) fragments lacking histidine (His) and Trp residues were tested for their capacity to protect from copper toxicity. A PrP(59-91) peptide lacking His residue shows as much neuroprotection as the native peptide; however, PrP(59-91) without Trp residues only partially protected against copper toxicity. The neuroprotective effect not only occurs with PrP(59-91), in fact a full neuroprotection was also observed using just one octamer of the N-terminal region of prion protein. We conclude that the N-terminal tandem octarepeat of the human PrP(C) protects neurons against copper toxicity by a differential contribution of the binding (His) and reducing (Trp) copper activities of PrP(59-91). Our results are consistent with the idea that PrP(C) function is related to copper homeostasis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Astrocytes / drug effects
  • Astrocytes / pathology
  • Copper / toxicity*
  • Hippocampus / drug effects
  • Hippocampus / pathology
  • Hippocampus / physiology
  • Injections, Intraventricular
  • Male
  • Maze Learning / drug effects
  • Memory / drug effects*
  • Molecular Sequence Data
  • Neurotoxins / pharmacology
  • Peptide Fragments / chemistry
  • Peptide Fragments / pharmacology*
  • Prions / chemistry
  • Prions / pharmacology*
  • Rats
  • Rats, Sprague-Dawley
  • Space Perception / drug effects*

Substances

  • Neurotoxins
  • Peptide Fragments
  • Prions
  • prion protein (59-91)
  • Copper