Copper and the Prion Protein: Methods, Structures, Function, and Disease
Top Cited Papers
- 1 May 2007
- journal article
- review article
- Published by Annual Reviews in Annual Review of Physical Chemistry
- Vol. 58 (1) , 299-320
- https://doi.org/10.1146/annurev.physchem.58.032806.104657
Abstract
The transmissible spongiform encephalopathies (TSEs) arise from conversion of the membrane-bound prion protein from PrPC to PrPSc. Examples of the TSEs include mad cow disease, chronic wasting disease in deer and elk, scrapie in goats and sheep, and kuru and Creutzfeldt-Jakob disease in humans. Although the precise function of PrPC in healthy tissues is not known, recent research demonstrates that it binds Cu(II) in an unusual and highly conserved region of the protein termed the octarepeat domain. This review describes recent connections between copper and PrPC, with an emphasis on the electron paramagnetic resonance elucidation of the specific copper-binding sites, insights into PrPC function, and emerging connections between copper and prion disease.Keywords
This publication has 95 references indexed in Scilit:
- A reassessment of copper(II) binding in the full-length prion proteinBiochemical Journal, 2006
- The Affinity of Copper Binding to the Prion Protein Octarepeat Domain: Evidence for Negative CooperativityBiochemistry, 2006
- A Mechanism for Copper Inhibition of Infectious Prion ConversionBiophysical Journal, 2006
- Tissue Safety in View of CJD and Variant CJDCell and Tissue Banking, 2005
- The Crystal Structure of the Globular Domain of Sheep Prion ProteinJournal of Molecular Biology, 2004
- Common Structure of Soluble Amyloid Oligomers Implies Common Mechanism of PathogenesisScience, 2003
- No Superoxide Dismutase Activity of Cellular Prion Protein in vivoBiological Chemistry, 2003
- Computational studies of Cu(II)[peptide] binding motifs: Cu[HGGG] and Cu[HG] as models for Cu(II) binding to the prion protein octarepeat regionJBIC Journal of Biological Inorganic Chemistry, 2003
- Copper Binding to the N-Terminal Tandem Repeat Region of Mammalian and Avian Prion Protein: Structural Studies Using Synthetic PeptidesBiochemical and Biophysical Research Communications, 1995
- Nerve endings from rat brain tissue release copper upon depolarization. A possible role in regulating neuronal excitabilityNeuroscience Letters, 1989