Prions

Prions are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein.

They characterize several fatal and transmissible neurodegenerative diseases in humans and many other animals. It is not known what causes the normal protein to misfold, but the abnormal three-dimensional structure is suspected of conferring infectious properties, collapsing nearby protein molecules into the same shape. The word prion derives from "proteinaceous infectious particle".

The hypothesized role of a protein as an infectious agent stands in contrast to all other known infectious agents such as viruses, bacteria, fungi, and parasites, all of which contain nucleic acids (DNA, RNA, or both). 

  • Creutzfeldt-jakob disease (CJD)
  • Variant creutzfeldt-jakob disease (vCJD)
  • Gerstmann-straussler-scheinker syndrome
  • Fatal familial insomnia
  • Kuru

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