Prion diseases of humans and animals: their causes and molecular basis.

  title={Prion diseases of humans and animals: their causes and molecular basis.},
  author={John Collinge},
  journal={Annual review of neuroscience},
Prion diseases are transmissible neurodegenerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) and scrapie in animals. Prions appear to be composed principally or entirely of abnormal isoforms of a host-encoded glycoprotein, prion protein. Prion propagation involves recruitment of host cellular prion protein, composed primarily of alpha-helical structure, into a disease specific isoform rich in beta-sheet structure. The existence… CONTINUE READING
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