Portal:Viruses/Selected miscellany/5

Prion protein in its properly folded form

A prion is an infectious agent hypothesised to consist of protein. This is in contrast to viruses and other known infectious agents, which all contain one or both of the nucleic acids, DNA and RNA. Prions propagate by transmitting a misfolded protein state. The prion induces existing, properly folded proteins in the host to convert into the misfolded prion form. Abnormal protein aggregates called amyloids accumulate in infected tissue and are associated with tissue damage and cell death.

Prion variants of PrP are associated with the transmissible spongiform encephalopathies in mammals. Human prion diseases include Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, kuru and variably protease-sensitive prionopathy. Prion diseases of other mammals include bovine spongiform encephalopathy ("mad cow disease") in cattle, scrapie in sheep, and chronic wasting disease in deer. All known mammalian prion diseases affect the structure of the brain or other neural tissue. All are progressive, lack an effective treatment and are inevitably fatal. Proteins showing prion-type behaviour are also found in some fungi. Fungal prions do not appear to cause disease in their hosts.