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Prion

pathogenic type of misfolded protein
(Redirected from Prions)
Prions infect tissues and make them look spongy. This sample is from Mad Cow disease.

A prion is an infectious protein. The word is short for "proteinaceous infectious particles".

All known prion diseases in mammals affect the structure of the brain or other neural tissue. At present they cannot be treated and always end in death.[1]

Prions cause many forms of encephalitis, or brain disease, such as scrapie, Creutzfeldt-Jakob Disease (Bovine Spongiform Encephalopathy) and kuru. Prions work by changing the shape of proteins in the infected animals. While normal proteins have lots of alpha helices, or twisted parts, changed proteins have lots of beta sheets, or flat parts. The word is pronounced pree-on.

Because prions are proteins, and proteins cannot reproduce themselves, it is a mystery how prions work. A lot of molecular biology research is going on to find out how they reproduce themselves, with no clear result so far.

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ReferencesEdit

  1. Prusiner SB (Nov 1998). "Prions". Proceedings of the National Academy of Sciences of the United States of America 95 (23): 13363–83. doi:10.1073/pnas.95.23.13363. PMC 33918. PMID 9811807.