Eli5: Been hearing a lot about Prions recently, what is it that makes them so terrifying?

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I’ve not heard about them much until recently, where i keep seeing mention of them, what exactly are they?

In: Biology

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Prions are proteins, large complex and weirdly shaped organic molecules that your body manufactures. There are a huge variety of different proteins, each serving specific functions in the body.

A prion is when a specific protein is misfolded in a specific way. This protein is non-functional now, but the issue is its very presence causes other proteins of the same type to misfold in a domino effect. This mass misfolding causes the proteins to stop doing whatever important job they had, and often causes the death of cells associated with them, leading to a slow and often unpleasant and incurable decline and eventual death of the afflicted creature, especially as prions most often cause the brain to break down.

Spontaneous occurrence of prions is ridiculously rare, so most cases of prion diseases are through transmission. In some prion diseases you need to physically eat the afflicted animal or even more specifically eat its brain to contract the disease, or in other prion diseases the afflicted animal can shed prions into the outside world, which can stick to stuff like plant leaves and get eaten along with the leaves later. What’s really concerning is prions are super tough compared to stuff like viruses, they can last for many years outside in the harsh elements and most of our techniques for sterilizing surfaces, food, and equipment of harmful microbes and viruses don’t even damage prions.

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