Abstract
The transmissible spongiform encephalopathies have presented a challenge to physicians and scientists attempting to develop immunologically-based treatments. Self-tolerance has been one of the major obstacles to successfully raising antibodies against the prion protein (PrP), the host-encoded protein whose misfolded form (PrPSc) is linked to the protein-only infectious agent responsible for these disorders. Recently, it has been shown that antibodies directed against the normal cellular isoform of PrP (PrPC) can reduce or eliminate PrP isoform conversion in both invitro and invivo model systems. Similar studies with a PrPSc-specific epitope target are in progress. There is now rational hope that this devastating group of diseases may soon be amenable to immunotherapy and immunoprophylaxis.