Transmissible spongiform encephalopathy (TSE) is thought to result from the structural conversion of cellular prion protein, PrP(C), into a misfolded oligomeric form, PrP(Sc). PrP is a cell membrane GPI anchored glycoprotein that is expressed in most tissues and in high levels in the nervous system. Some likely cellular functions of PrP are (1) cell protection against oxidative stress, (2) induction of neuronal cell adhesion, neurite extension and maintenance, and (3) neuroprotective signalling through a cAMP/PKA-dependent pathway.
