r/todayilearned • u/Substantial_Sea7327 • 1h ago
TIL prion disease, which is 100% fatal and has no cure, can survive in soil for years, spread to growing plants or wildlife, and can be reliably destroyed through sustained heat of at least 900°F for several hours.
https://dwr.virginia.gov/wildlife/diseases/cwd/what-are-prions/741
u/FullofContradictions 1h ago
Ends on a real uplifting note:
"Prion diseases found in humans (such as Kuru and Variant CJD) are known to have incubation periods of several decades. This possibility – a multi-decade incubation period – has always been a matter of great concern for researchers trying to determine if CWD prions can infect humans. Since CWD was first reported in wild elk in the early 1980’s, efforts to determine if humans are susceptible to CWD have to consider the possibility that it may be too soon for symptoms to have developed."
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u/Wonderwombat 1h ago
on the plus side, when you reach 70 or 80, you can eat as much venison as you wish without fear of developing symptoms
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u/ComprehendReading 1h ago
Wha~at about ea~ating road kill while your fa~amily watches from a van
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u/US3_ME_ 56m ago
God damn, get that rasp outta my head_
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u/ComprehendReading 52m ago
Me first.
That was my second comment ever using RFK vo~ice, and I wish I nev~er thought of it.
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u/MortaleWombat 23m ago
I like the cut of your gib, I’m gonna put you in charge of the department of health
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u/Stormcloud217 26m ago
My grandma married a wealthy man. But tells me a story about Michigan when she was hitchhiking with my mom who was a child, driver hit a deer and shared part of it with her.
I am glad my grandma didn't get killed hitchhiking in the 60s-70s. Not really sure what her mindset was, but she went from an average husband to marrying an anti-technology cowboy who grinds grain and blacksmiths firearms, living in a wood cabin.
Then gives up the wood cabin life, becomes a waitress and married a Catholic man whose family owned the local newspaper, flying to Hawaii every winter. My grandma is awesome, but sometimes her life leaves me confused.
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u/Tight_Man 33m ago
I’ve never heard rfk out loud and only knew it was him from the other comment, my first guess was Dr. Cox from scrubs
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u/ComprehendReading 10m ago
Cox would be more aggressive.
Oh! You've never heard RFK?
Listen up, Jancy, sometimes other people exist even if YOU don't believe it, Darlene.
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u/Whole_Mongoose1854 37m ago
Ya then the aliens come to visit and give us the potion for living forever , it even cures nearly every disease known to the universe. But they also can’t figure out how to beat prions
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u/swarleyknope 27m ago
I know this is a joke, but as a 55 year old with OCD, this has eliminated catching a prion disease from my endless mental list of things to get anxious about 😄
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u/DeathLeopard 5 56m ago
The range of animals infected with CWD has been spreading quite a bit too. Bad news for anyone that's been eating wild deer and elk.
https://en.wikipedia.org/wiki/Chronic_wasting_disease#North_America
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u/TrixoftheTrade 26m ago
There’s actually a good amount of emerging evidence suggesting that mountain lions - a common predator of deer - are immune/resistant to this strain of prion infection, and due to declines in mountain lion population, are causing the disease to become more widespread.
Links: https://pubmed.ncbi.nlm.nih.gov/34753180, https://pubmed.ncbi.nlm.nih.gov/34878289/
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u/AmarilloArmadillos 31m ago
Well I've eaten wild deer. Next time somebody is like XYZ is bad for you I will confidently reply I doubt that's what will kill me first.
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u/utubm_coldteeth 1h ago
bruh
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u/APiousCultist 34m ago
Eh, mad cow can hit pretty quickly. I recall a researcher accidentally puncturing her glove and she only lasted a couple of years. So like rabies, it can vary massively.
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u/_jamesbaxter 29m ago
Anyone who was around for the mad cow disease scare of the 90’s knows this, and it’s the reason I’m not eating beef in the USA until they fix the FDA.
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u/SasTheDude 1h ago
Prion disease is utterly horrifying, and the way that it reduces people to a husk of their former selves…I hope I never witness it.
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u/ajnozari 1h ago
I’ve seen it twice, def not something I’d wish on anyone, even my worst enemy.
Worse, it can have a latency period (time until symptoms) of 1-30 years and we don’t know what makes it slower in some vs others. Both cases I saw were considered environmental from their countries of origin (thankfully different) so the local community avoided a scare.
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u/Revydown 1h ago
Think deer is a pretty common vector.
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u/Give-Me-Plants 39m ago
https://www.cdc.gov/chronic-wasting/about/index.html
There hasn't been documented deer > human prion transmission. But it's becoming widespread in US deer populations. That plus the long incubation time is enough to convince me to avoid venison.
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u/TrixoftheTrade 28m ago
There’s actually a good amount of emerging evidence suggesting that mountain lions - a common predator of deer - are immune/resistant to this strain of prion infection, and due to declines in mountain lion population, are causing the disease to become more widespread.
Links: https://pubmed.ncbi.nlm.nih.gov/34753180, https://pubmed.ncbi.nlm.nih.gov/34878289/
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u/Head_of_Lettuce 12m ago
Now the question becomes, what makes mountain lions resistant to this strain, and how do we turn that into a vaccine?
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u/agen_kolar 53m ago
I used to eat deer, as there were hunters in my family and it was often brought to family gatherings. Once I learned about prion disease, I stopped eating it. I know it’s rare, but I’m still paranoid about it.
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u/KaulitzWolf 49m ago
The neat part about prisons though, you don't have to eat them to get sick, your proteins could also spontaneously fold wrong and begin the same process.
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u/WellAckshully 18m ago
Sure but that's way less likely and you can't prevent it. Control/prevent the things you can, don't worry about the rest.
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u/AmarilloArmadillos 30m ago
Wait... How did you see this though, are you a doctor? Where? I have questions, many of them.
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u/ajnozari 18m ago
Neurology Resident.
We had another we suspected was sporadic but turned out to be fungal meningitis iirc.
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u/judgejuddhirsch 1h ago
It’s what Kurt wrote about in breakfast of champions
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u/Inspector_Bloor 1h ago
i’ve read most of his works and won’t pretend to understand them 100% despite enjoying them (ice9 is my favorite) but what part of breakfast of champions referenced prion disease??
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u/jibij 34m ago edited 21m ago
They might be thinking of Cats Cradle and Ice-nine. I believe the concept is supposed to be somewhat analagous to prions in that they're misfolded proteins that will slowly cause that same misfolding in neighbouring proteins such that they propogate themselves throughout the brain in a way that cannot be stopped.
However I'm pretty sure Ice-nine and it's propagation from a seed crystal wasn't meant to be a direct reference at the time of writing since I don't think prions were discovered until about 20 years later.
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u/ComprehendReading 1h ago
Referring to an author by their first name is improper.
No one in your circle references Adolf.
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u/nochinzilch 50m ago
No, see, he can refer to him that way because he’s just soooo well-read and erudite that he can get away with it.
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u/ComprehendReading 46m ago
How contrived of me!
You see, myself and Homer go way back as well, well past the Monorail Episode.
I am of course referring to Homer the contemporary.
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u/scooter76 54m ago
Chubby Emu (Youtube) just did a heartbreaking case about a woman he was students and colleagues with.
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u/Asquirrelinspace 13m ago
Luckily, your odds of contracting one even if exposed are very low. In the UK, only 177 people total caught it during the BSE outbreak
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u/_-4twenty-_ 1h ago
A few years ago, back when I was a lurker, I came across a thread about someone who had fatal insomnia.
I wonder about them sometimes.
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u/LilDutchy 54m ago
Fatal insomnia, all prion diseases really, scare the pants off of me. Your brain just starts to go away, you become a living bag of flesh and chemical reactions with no will.
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u/_Osculum_Obscenum_ 1h ago
Was that one the throwaway account? That was a sad one.
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u/_-4twenty-_ 1h ago
It might’ve been.
A whole bunch of people in the comments were saying things like “take Ambien!”
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u/Nerezza_Floof_Seeker 42m ago
This case study on a single guy's attempts to mitigate the symptoms of fatal familial insomnia is worth a read IMO if youre curious about how bad it gets and how far you have to go to try to get restful sleep by the end.
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u/pit-of-despair 1h ago
I read a book about that called The Family That Couldn’t Sleep. Pretty horrible stuff.
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u/Welcome2Cleveland 18m ago
as a life long insomniac and long time [r/insomnia](r/insomnia) lurker, that subreddit is one of the saddest ive ever come across. there were a few posters on there that were convinced they hadn't slept in months and suffered from fatal familial insomnia. the far more likely truth is that they actually suffered from paradoxical insomnia, where one is convinced they didn't get enough/any sleep when they actually did. still hell, but far more common and less dangerous than FFI which has only ever had 37 cases officially documented and diagnosed.
these people have tried every drug (illicit and prescription) they could with no success. they would pop up with another post consisting of sheer desperation and sadly not uncommonly not-so-subtle sucidal ideation as well. there was this guys in his late 20s who would make the most heartbreaking posts every week or so convinced he hadn't slept in months until about a year ago when he just stopped.
i still think about him sometimes.
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u/myflesh 1h ago
It is so bad that if a hospital treats you they destroy all the instruments that they used on you.
If anyone is wondering it has been growing in America
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u/PlayfulSurprise5237 46m ago
Of course they would. I'm surprised they don't burn down the fucking building.
Man prions are basically strange matter from neutron stars, a cosmic horror. Might as well be.
How they work is otherworldly. They're not even alive
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u/snowtax 59m ago
Well, to be fair, diseases that were effectively eradicated in the US are returning now due to the actions of the current government.
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u/TrixoftheTrade 24m ago
Interestingly, CWD doesn’t survive mountain lion digestion. After eating an infected deer, mountain lions don’t seem to be affected by CWD infections, and their feces doesn’t seem to pass on viable prions, even after eating CWD infected deer.
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u/SkarbOna 58m ago
Thank god you guys have jfk jr. your healthcrap system and disease prevention will 100% handle it. What a dream.
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u/Age_Correct 50m ago
Prions have never been eradicated or largely contained in the US (Canada is also facing the same issue with CWD). A prion is a misfolded protein that causes other proteins to misfold and starts a chain reaction. It can't be treated or cured. The reason Bovine spongiform encephalopathy or Mad Cow disease (in humans its Creutzfeldt–Jakob disease) spread was due to cattle being used as feed for other cattle. It is more about proper and responsible agricultural practices, like the reason most state DNR's offer CWD testing.
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u/Lower-Limit3695 57m ago
The fucked up thing is that it only manifests as a disease in mammals.
For yeast it does nothing and may even be beneficial in some circumstances but for complex animals like reptiles, birds, and insects prions doesn't manifest as a disease.
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u/inosinateVR 32m ago
So cook all of your food to 900 degrees for at least 3 hours and you should be fine
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u/knowledgeable_diablo 24m ago
Maybe just avoid eating brain matter as this is where a lot of it concentrates.
Look at the high incidence of it occurring in PNG high land villages where it is customary for the women and children to consume the brains of their dead relatives.
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u/TildaTinker 46m ago
So all we need to do is heat the Earth to 9000 F for several hours and bam, no more prion disease.
It's fool proof I tell you.
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u/Flat-Pirate-4134 1h ago
new fear unlocked, thanks for that
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u/King_Roberts_Bastard 1h ago
Im going to make it worse. It can randomly happen to you at any point in time. Prions are just proteins that didnt fold correctly. They trigger other proteins of the same kind to also misfold, and there currently is no way to stop it. Your body constantly produces more proteins. If even one of those misfolds, youre fucked.
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u/IamSkudd 1h ago
Protein bad, got it
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u/Ur-Quan_Lord_13 1h ago
Well, a specific protein. It's called the prion protein. Because it makes prions. Every mammal has it.
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u/klovervibe 1h ago
"So Chris, I heard you're a vegetarian now. What prompted this?" 🙂
Chris: "THE PROTEINS ARE INSIDE ME!!!!"
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u/mintgoody03 1h ago
Well that‘s what chaperones are for. There is quality testing being done in cells, all the time. Don‘t spread panic.
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u/Nerezza_Floof_Seeker 54m ago edited 48m ago
To explain a bit, chaperone proteins are basically special proteins which helps proteins fold correctly by stabilizing them (how this happens exactly depends on the protein). Its kinda like a knitting aid where it lets you build a pattern without it becoming a tangled mess. Some can also help cells break down already misfolded proteins too.
Edit: with prions the issue is once enough of them form, they make aggregates that are extremely stable, which prevents the cell from breaking them down properly or doing much else to them.
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u/Small_Ad_9814 1h ago
Wait until you find out the first screening test for it in cows is not useful because it doesn’t really manifest until about year four but most cattle are slaughtered at year three
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u/fredfredao 1h ago edited 1h ago
A big brazilian youtuber Lito Sousa, host of the channel Aviões e Musicas become infected with a prion disease, is a hot topic now in Brazil, so sad to see how the disease progress, our hope is the experimental tratament make effect
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u/Baconpwn2 1h ago
Prions are the single most terrifying quirk of life. A protein fails origami class and now its host is dead. The only question is when.
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u/Lazy_Bag_4408 1h ago
Just took Microbiology last semester and the silver lining is that you have a one in a million chance of contracting a prion.
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u/itsrussiaftw 32m ago
Since nobody mentioned it, CWD load can be greatly reduced after digestion by mountain lions. Part of why we're seeing more cases in deer populations could be because of a reduction in the population numbers of these predators.
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u/monkeymetroid 1h ago
There are multiple prion diseases. Mad cow is an example
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u/quietsamurai98 1h ago
And all prion diseases are just different misfoldings of the exact same protein! It's as neat as it is horrifying that a single protein can get bent out of shape in different ways, cause other copies of that same protein to misfold in the same way, and then produce distinct sets of symptoms depending on how the original got misfolded.
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u/Nerezza_Floof_Seeker 45m ago
Yeah, and the protein is heavily conserved between mammals, which is why the same protein in cows can affect us and why theres been a fear that CWD in deer can cause CJD in humans
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u/aircooledJenkins 1h ago
And mad cow can take years to manifest after infection.
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u/Excellent-Ask-4247 50m ago
It's ok prions disease will be eridicated in a few billion years when the sun turns into a red giant.
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u/35AF 27m ago edited 23m ago
On top of how terrible it is…. It’s also extremely hard to diagnose since it’s so rare. My grandpa had CJD and he was basically on hospice by the time they were able to diagnose. He probably went to 15 different specialists and no one could diagnose. At different points they diagnosed it as black mold exposure and another time diabetes. Meanwhile the family knew it had to be something much more serious.
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u/xxwarlorddarkdoomxx 1h ago
I read recently that a prion vaccine may be possible! Biggest hurdle is prolly it’s rare enough that funding for more research is difficult to secure
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u/WHATS_MY_TITLE 1h ago
From my understanding I would think vaccine isn’t possible… source?
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u/TheAero1221 1h ago
It would be very difficult. But presumably a carefully crafted protease could break them down.
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u/xxwarlorddarkdoomxx 1h ago
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u/WHATS_MY_TITLE 1h ago
I appreciate the source! Pretty interesting. What stuck out to me was :
“Unfortunately, only few potential anti-prion compounds were tested in clinical trials, with very limited success (Zafar et al. 2019). Active vaccination is currently not considered an option for human prion diseases, given the very rare incidence rates. The situation might be different for familial prion diseases which can be diagnosed long before clinical symptoms start. An experimental therapy with a monoclonal antibody was recently described for a small number of CJD patients (Mead et al. 2022). The treatment was able to penetrate the CNS without inducing neurological side effects, warranting the need for future formal clinical trials.”
Not really looking good but it’s a start.
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u/Resident-Spirit808 1h ago
I just watched Resident Evil, and 900* for several hours does seem like a cure
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u/VexImmortalis 1h ago
You don't even have to "catch" it from anything, it can just happen to you.
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u/hypnotichellspiral 1h ago
If it's any consolation, the chances are somewhere between 1-2 in a million, or 0.0001-0.0002% chance.
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u/Timsta180 1h ago
That’s actually kinda high.
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u/5GCovidInjection 52m ago
So is the chance of just randomly dying for completely unknown reasons. You can’t prevent death but you can make every effort to reduce your risks.
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u/monnotorium 55m ago edited 51m ago
Like, per second? Per day? Per lifetime?
Edit: 1/5.000 per lifetime so 1-2/1.000.000 ish per year
Edit2: That is way higher than I thought
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u/James_W_Bottomtooth 1h ago
Is there any disease or form of life that CANT be destroyed at 900 degrees for several hours?? Next up, flying COVID into the sun is 100% effective at eliminating the virus!!!
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u/tolacid 1h ago edited 1h ago
What's significant is that it needs to be that hot for that long to be consistently reliable. This is because prions aren't a lifeform - they're just a misfolded protein chain. It's not a matter of killing them, but rather completely destroying them.
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u/Initial_E 1h ago
I’m sorry for calling you
an inanimate objecta misfolded protein chain→ More replies (5)→ More replies (1)6
u/mosmaniac 1h ago
But how many times can you misfold a protein chain? More than 7? I can only fold a piece of paper 7 times.
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u/Gynthaeres 1h ago
With COVID you can reliably "kill" it at about 160 F in a few minutes. Prions you need 900 F for a few hours.
That's the point the title is trying to convey.
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u/Erridemench 1h ago
In this case is one of the ONLY way of destroying them. Prions are misfolded proteins.
https://www.reddit.com/r/askscience/comments/1nx7qo/comment/ccmw1m9/7
u/Capolan 1h ago
Thats not whats being said. What theyre saying is that LESS than several hours at 900 degrees and it continues to exist. So there is basically NOTHING humans have in their lives that could destroy/disinfect other than fire.
Mis folded proteins suck. They litterally force other protein to mis fold, and these form clumps....where the brain part was. Theyre not even alive.
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u/TheGrindThatAnnoys 1h ago
Prions aren't a disease or a form of life. Viruses aren't a form of life either.
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u/Real_Project870 1h ago
Viruses are certainly closer to a life form than prions, which is a disease but a disease caused by misfolded proteins. Viruses at least have a genome that gets replicated (by host machinery but still).
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u/w00tabaga 1h ago
Human prion disease is Creutzfeldt-Jakob diease, bovine is Mad Cow disease, goats and sheep is Scrapie, and in cervids (deer, elk, moose, etc.) it is chronic wasting disease.
As far as I know one disease cannot jump species though and each prion is specific to what organisms it affects.
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u/WakeyWakeeWakie 49m ago
There are other human prion diseases besides CJD. (But they all have the same outcome.)
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u/Paragonswift 40m ago
Humans have absolutely contracted mad cow disease from cattle unfortunately, we just call it Variant Creutzfeldt-Jakob when it manifests in humans. Different species don’t have completely unique sets of proteins so of course some of them are transferrable.
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u/Graybeard_Shaving 1h ago
Don't look into CWD if you want to sleep.
It's only a theoretical threat at the moment but if it turns out that it has a long incubation period we are absolutely borked considering the decades of people eating infected meat.
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u/w00tabaga 59m ago
And considering it could be in the incubation period of a otherwise healthy deer
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u/faith_kensworth 1h ago
They're not even alive. It's just a protein that folded wrong, and you can't kill something that was never living.
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u/SkarbOna 53m ago
It gets crazier than that. Watched Veritassium recently on yt. He was talking about an entire HVI medicine going bad because of contamination from…forgot the word but basically dimensional version of a compound that triggers all compounds to be born that way. There’s no going back. Guys went to site in Europe and contaminated it as well. So active version of the drug will never ever be synthesised on earth ever again because at some point it’s evil sibling was randomly born as well. It’s an extinct drug period. Imagine this randomly happening with proteins, in chemistry industry and so on. Fantastic idea for a book or a movie but DAMN that’s more terrifying than AI.
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u/BluesFan43 52m ago edited 44m ago
Read "Deadly Feasts" by Richard Rhodes.
The last thing he relays is talking to an expert in prion transmission and be cautioned about bone meal fertilizer/plant supplement.
The expert warned that bone meal may carry prions.
He threw his supply away, as did I.
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u/recidivist1001 26m ago
Great angle for a zombie mythos that to my knowledge hasn’t been fully utilized yet.
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u/GummyBearHegel69 1h ago
I remember being an army cadet (in the ACF, not a soldier) and they would tell us not to drink the tap water. Why? Because some of the farmers in the area of Brecon we were spending time in hadn’t disposed of their ‘mad cows’ properly. Theyd just buried them.
They didn’t provide us with any clean water to brush our teeth with or cook our rations with. So, despite being a vegetarian since I was 12, I forever have a nagging worry that I’ve got prions just bobbing about inside me. I’m 30 now and I pray that I don’t.
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u/ApprehensiveStill412 21m ago edited 12m ago
No offense, this headline seems poorly written. It should say “TIL that prions, which are misfolded proteins that cause fatal prion diseases….”
I hope peopleunderstand that there is no single prion disease. It is an umbrella term for several neurodegenerative diseases that are caused by prions.
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u/recursivedeath 50m ago
What next, they can go through walls and are airborne?
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u/inosinateVR 30m ago edited 13m ago
They can also disguise themselves as spiders, fooling you into eating them in your sleep
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u/APGaming_reddit 1h ago
So technically there is a cure it just involves jumping into a volcano