Prions are infectious agents made up of misfolded proteins that can reproduce by misfolding normal healthy proteins. They are responsible for transmissible spongiform encephalopathy diseases, including mad cow disease. Prions found in animals only infect the brain, are fatal and incurable. Prions have been discovered in fungi, but instead of harming them, it is theorized that they may play a symbiotic role in the fungi’s immune system. The mechanism of reproduction among prions is not yet fully understood.
A prion is a non-living infectious agent made up of misfolded proteins. It can reproduce by misfolding normal healthy proteins with the help of its host’s biological mechanism, like a virus. “Prion” is short for “infectious protein particle”.
The idea of a nucleic acid-free infectious agent for reproduction was initially met with skepticism when the idea was proposed in the 1960s, because such a phenomenon had never been observed before. Although nucleic acid-based entities are well known, the mechanism of reproduction among prions is not yet fully understood. Prions found in animals only infect the brain, are fatal and incurable. Prions have been discovered in fungi, but instead of harming them, it is theorized that they may play a symbiotic role in the fungi’s immune system.
Prions are responsible for the mad cow disease epidemic in Britain during the early 00s, which led to the destruction of thousands of livestock, and international fear that the disease could spread from cattle to humans. Prion protein was not isolated until 1982, when Stanley B. Prusiner discovered it and coined the term. He received the Nobel Prize in Physiology or Medicine in 1997 for his discovery.
One of the first cases of prion disease to be investigated was scrapie, a disease affecting sheep and goats. This disease had been known since the 18th century, but it wasn’t until the 1918s that scientists began to suspect scrapie was not a typical disease, reproducing outside the normal means of nucleic acids. Scrapie, like most prion diseases, is fatal to the animal it infects, causing horrific symptoms before death. The disease’s name comes from the eerie behavior of infected sheep, of scraping its fleece against rocks. The prion also causes inability to walk, seizures, and excessive lip smacking.
Prions are responsible for diseases included in the class known as transmissible spongiform encephalopathy. (The scientific name for mad cow disease is bovine spongiform encephalopathy.) The disease is so called because it causes the victim’s brain to degenerate into a spongy consistency.
Most prions are not transmissible between species, but there have been reports of humans contracting a version of the disease called vCJD, with 4 deaths occurring in the town of Armthorpe in the UK. Reports like these have led to the worldwide terror that accompanies mad cow disease. Since the outbreak, several dozen cases of prion infection have been reported in a variety of countries around the world, such as Japan, but many large livestock-producing countries remain disease-free. The US meat industry suffered terribly when news of the disease hit American shores, although the likelihood of a bovine prion infecting a human is staggeringly small.
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