Encyclopedia of Metalloproteins

2013 Edition
| Editors: Robert H. Kretsinger, Vladimir N. Uversky, Eugene A. Permyakov

Copper and Prion Proteins

Reference work entry
DOI: https://doi.org/10.1007/978-1-4614-1533-6_102

Synonyms

Definition

Prion diseases are infectious, neurological disorders that arise from accumulation of a misfolded form of the endogenous prion protein. These disorders affect a wide range of mammalian species and present an ongoing public health threat. The function of the normal prion protein is not well characterized, but recent research suggests that it is linked to the protein’s ability to bind copper.

Prions, Copper Coordination, Function, and Disease

The prion protein, discovered by Stanley B. Prusiner and colleagues, is a ubiquitous component of tissues in both mammals and avian species that is found at high levels in the central nervous system (CNS). The normal form is referred to as cellular PrP and denoted PrP C. PrP C is a glycoprotein of approximately 210 amino acids, with an unstructured N-terminus and a folded C-terminus containing three α-helices (Fig.  1). The final...
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References

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of Chemistry and BiochemistryUniversity of California, Santa CruzSanta CruzUSA