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

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Summary

Introduction

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

How Do Prions Enter the Body?
How Do Prions Damage the CNS?
Do Mammals Have an Antiprion Defense System?
How Can the Prion Problem Be Resolved for Good?
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