Prions are nightmare fuel. It's surprisingly not as much talked about despite the damage they can cause.
- There is not cure or prevention
- can linger in soil, on trees, and on hunting bait for years or decades
- really hard to detect, since it takes years to show the symptoms
- induce behavior where prions get to spread more
- potential to jump species, including primates
it's because it's basically a quirk of one protein, which is only really found in the brain and nervous system, which is well isolated from the environment. And while it's resilient it's not like it grows in any environment which isn't the brain. So while it's a big problem if it gets in, it's really hard for it to happen in normal circumstances (even eating it, which is the main vector in animals, requires you eat quite a lot or get ludicrously unlucky).
Put wolves at charge of solving the problem. They seem to be immune to the prion and are extremely good keeping deer populations healthy. Is what they do.
This is the result of having crowded game farms, basically breeding deer like rabbits and accumulating them on small areas so people can come and have fun shooting.
I would assume the only way to counteract this would be to come up with some sort of counter folded protein that we would have to continually take and is always present in the blood before infection. As soon as the prion hits, it would combine with one of these and become harmless. Maybe with enough time we could come up with a genetically engineered way to have our immune systems create this counter folded protein.
> the molecules can linger in soil, on trees, and on hunting bait for years or decades.
Given the proliferation of biological life, I assume that there's some scavenger, decomposer, or abiotic process that is notable in destroying proteins lying around...
Are these prion-proteins unusually durable, or is it about average?
Anyone has an insight on why all these prion-borne diseases ultimately destroy the brain in similar ways? Do they all target the same family of proteins, only slightly different depending on the species? Are there prion diseases that affect other organs?
My understanding is that all the well known mammalian prion diseases are the same prion protein. The disease it causes has a variety of names in different contexts: CWD, vCJD, kuru, mad cow.
There are some other suspected prions in fungi, and there's suspicion that some other human diseases are actually caused by an as-yet-unknown other kind of prion that affects a different protein, but the well-studied diseases are all misfoldings of essentially the same protein.
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- There is not cure or prevention - can linger in soil, on trees, and on hunting bait for years or decades - really hard to detect, since it takes years to show the symptoms - induce behavior where prions get to spread more - potential to jump species, including primates
This is the result of having crowded game farms, basically breeding deer like rabbits and accumulating them on small areas so people can come and have fun shooting.
Maybe a job for Alphafold?
Given the proliferation of biological life, I assume that there's some scavenger, decomposer, or abiotic process that is notable in destroying proteins lying around...
Are these prion-proteins unusually durable, or is it about average?
https://pubmed.ncbi.nlm.nih.gov/34878289/
but yes they are extremely challenging to destroy
There are some other suspected prions in fungi, and there's suspicion that some other human diseases are actually caused by an as-yet-unknown other kind of prion that affects a different protein, but the well-studied diseases are all misfoldings of essentially the same protein.
https://en.m.wikipedia.org/wiki/Prion
There has been one other prion candidate found that essentially shreds every other organ system, in fact.
https://en.m.wikipedia.org/wiki/Alpha-synuclein
Decontamination and denaturing of the bloody things requires going to Herculean lengths.
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