Abstract
Prion diseases are characterized by deposition of PrPSc, a misfolded and aggregated isoform of the host-encoded cellular prion protein (PrPC), within the central nervous system (CNS) and other organs. Here we review the current knowledge on five issues relevant to prion diseases: (1) how do prions enter the body, (2) how do prions reach the central nervous system, (3) how do prions damage the CNS, (4) do mammals have an antiprion defense system, and (5) how can the prion problem be resolved for good.
Highlights
Prion diseases are characterized by deposition of PrPSc, a misfolded and aggregated isoform of the host-encoded cellular prion protein (PrPC), within the central nervous system (CNS) and other organs
Most cases of human prion disease occur for unknown reasons, and .20 mutations in the prion gene (PRNP) may lead to inherited prion disease
Prion diseases are contracted by exposure to prion infectivity
Summary
Prion diseases are characterized by deposition of PrPSc, a misfolded and aggregated isoform of the host-encoded cellular prion protein (PrPC), within the central nervous system (CNS) and other organs. We review the current knowledge on five issues relevant to prion diseases: (1) how do prions enter the body, (2) how do prions reach the central nervous system, (3) how do prions damage the CNS, (4) do mammals have an antiprion defense system, and (5) how can the prion problem be resolved for good
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