The Enigmatic Multifunctionality of the Prion Protein.

There is conclusive evidence that the host gene encoding the prion precursor protein (PrPc) is implicated in the development and propagation of transmissible spongiform encephalopathies collectively known as prion diseases. Nevertheless, the normal cellular function of this widely expressed and highly conserved gene product remains elusive. Here we review evidence implicating PrPc in a number of diverse phenomena including the transportation and metabolism of metal ions associated with protection against oxidative stress; behavior as a membrane receptor or ligand, or a receptor-bound molecule implicated in signal transduction; and as a nucleic acid-binding protein with the functional properties of a nucleic acid chaperone protein. A complex picture is emerging of PrPc as a multifunctional protein. (c) 2002 Prous Science. All rights reserved.