Central to Prion diseases is the normal endogenous Prion protein, PrP C. In spite of years of research the exact function of this protein remains enigmatic. Numerous binding partners have been identified for PrP C and due to the presence of a repeated sequence of PHGGGWGQ in the proteins amino-terminus it can bind metal ions. The protein is a complex molecule and each portion of PrP C possesses different roles for function and/ or trafficking. As understanding the role of PrP C is central to these disorders the structure/function relationship will be reviewed here.
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