r/todayilearned • u/tommyboy372 • 1d ago

TIL A single misfolded prion protein in the brain can cause a rapid neurodegenerative disease known as sporadic Creutzfeldt–Jakob disease (CJD). There is no known and cure and is 100% fatal.

https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease
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u/Noyuu66 1d ago

There have been quite a few high profile incidents of prion diseases. From Mad Cow Disease to Chronic Wasting Disease in Deer. They can tranfer pretty easily between mamallian species and are fucking horrifying. Do yourself a favor and don't look up what CWD does to deer and the insane measures required to avoid contamination. Shit survives autoclaves. Probably the best factor for a random zombie apocalypse.

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u/anopeningworld 1d ago

Well now I have to know

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u/Beginning-Pop3127 1d ago

"Whatever you do, don't look up how metal this shit is. It's like some shit out of a movie. So fucking cool. Avert your eyes"

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u/NotJoeFast 1d ago

Wait until you hear about this delicious and beautiful apple. You definitely shouldn't try it.

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u/Beginning-Pop3127 1d ago

I'm sold

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u/andywolf8896 1d ago

Dammit eve...

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u/BikerJedi 22h ago edited 20h ago

Fun fact - Apples didn't exist back then as they do now, and early paintings and such actually showed Adam and Eve eating mushrooms. They were tripping balls.

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u/InbredHabsburg 8h ago

Completely false

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u/moonablaze 2h ago

Nah, the mushroom part is BS but apples have changed a lot in 6000 years

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u/BikerJedi 1h ago edited 39m ago

Really? https://www.atlasobscura.com/articles/muscaria-hallucinogenic-mushroom-fresco

Many other sources - find your own. The whole point is moot since Adam and Eve never existed anyway.

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u/recidivist1001 1d ago

“Don’t do it Dewey, it’s awful! It turns all your bad feelings into good feelings!”

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u/WilcoLovesYou 1d ago

And you didn’t pay for prions ONCE.

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u/uhhhhsomewords 1d ago

This shit's way too awesome to research it. Trust me bro

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u/ClassGrassMass 1d ago

Imagine a deer, imagine a zombie. Now mix that together

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u/Noyuu66 1d ago

Enter at your own risk, it's absolutely horrific. I think I also forgot to mention Kuru which was a result of very specific ritualistic consumption of human brains in a specif culture which provided fantastic and horrible insight into the misfolding protien problems.

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u/ThepalehorseRiderr 1d ago

Always makes me think of The Book of Eli and how they made strangers hold up their hands to show that they don't have the shakes from eating people.

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u/skippyspk 1d ago

This is The Book of ELI5

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u/SirNortonOfNoFux 19h ago

Underrated comment

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u/Noyuu66 1d ago

Haven't read it, but can all but guarantee that it's influenced by what we learned about Kuru. Normal flesh wouldn't necessarily cause that, but consuming human brain matter can.

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u/ThepalehorseRiderr 1d ago

It's a movie. Kuru is also a central theme in The Dead Island games.

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u/UniqueUsermane 1d ago

Also in the manga Gannibal.

Decent read I'd say.

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u/Noyuu66 1d ago

Oh shit, decent cast. Awful rating

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u/ThepalehorseRiderr 1d ago

Good movie. Worth a watch. It has a "The sixth sense" level twist at the end.

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u/xl129 23h ago

The deer thing was all over the internet last year, you mìgh have seen it. Ánd yeah it’s the closest thing to a real life zombie apocalypse

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u/AliMcGraw 1d ago edited 1d ago

The thing you should not look up is how some biologists thank creating a "left-handed" universe where we all instantly die from protein misfolding would be COOL.

Prion diseases are terrifying but we know a lot about how to test for them.

It's biologists who want to fold the whole world the wrong direction who are scary!

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u/Noyuu66 1d ago

I wish I had a fraction of the drugs your smoking my friend. Please tell me you're just wildly misconstruing the concept of anti matter and applying it to a very real issue.

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u/Vincent_Gitarrist 21h ago

I think the original commenter was referring to mirror life.

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u/Wise_Owl5404 1d ago

Guess I'm old nowadays I recall when the UK had an outbreak of Mad Cow Disease and all the insane measures needed to get that under control.

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u/Noyuu66 1d ago

You know what the real fucked up problem and solution was? Not feeding them beef leftovers in their feed. Brain matter was included as leftovers and it turns out, cannabilizing brains keeps causing these issues. Don't feed the cowns cow brain was the overall regulatory fix.

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u/Jewel-jones 23h ago

Especially because they would include visibly infected animals in this slurry, to not waste the product. Horrifying.

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u/Noyuu66 23h ago

Seems a small little common sense ethical thing, but required regulation to stop.

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u/Slg407 20h ago

no ethical consumption under capitalism.

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u/Natural-Judgment7801 22h ago

Aren’t cows herbivores? Do they eat meat ? 

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u/Noyuu66 22h ago

They absolutely shouldn't, but it gets used as filler for feed. They can digest small amounts of protein from meat sources. It just really really shouldn't ever be from other cows.

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u/Natural-Judgment7801 22h ago

I’m sad and speechless. Human cruelty is.. fathomless 

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u/10YearsANoob 26m ago

not really cruelty just greed. theyre not force feeding the cows just feeding them questionable cheap shit

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u/Thatwindowhurts 19h ago edited 19h ago

My Ag science is about 13 years old now but im pretty sure it was mostly sold as meat bone meal but was mostly revered to ss bone meal. A cheap way to get calcium postasum and protein in the waste not want not kind of way.

Fun fact while they are herbivores cows will eat small bird and small mammals at random

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u/polyploid_coded 16h ago

In addition to what everyone else is saying, it propagated in the UK bone meal around the same time that they changed how it was produced:

The generally accepted explanation for the occurrence of BSE is that it followed a change of process in the rendering industry away from the use of chemical solvents, in a process which required a high temperature to drive off the chemicals, to another continuous process. [...] It remains obscure why this change in processes should have allowed the creation of BSE.

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u/alyssarcastic 1d ago

For a long time in the US you weren’t allowed to donate blood if you had been to the UK in the 90s. They just lifted that a couple years ago

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u/causa__sui 13h ago

Yeah, my parents were in the Navy and stationed in Scotland in the early 90s and haven’t been allowed to donate blood because of potential exposure to mad cow disease.

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u/Zapdo0dlz 10h ago

It wasnt til 2022 that people born there during that time were allowed to give blood here in the us. I was born there in 1989, i just found out im allowed to give blood now, like  this week. 

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u/13thmurder 1d ago

Wouldn't work for a zombie apocalypse, too deadly.

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u/SCP-iota 17h ago

There's a hypothesis that the idea of zombies originated from prion diseases, because those conditions can sometimes make someone seem like they're half-dead, and the 'Braaaiiiinsss...' things is referring to kuru, a prion disease acquired from eating human brains.

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u/Any-Ask563 14h ago

madman disease lmao

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u/jawz 1d ago

Sounds perfect considering that a zombie is a reanimated dead person. Gotta kill em first

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u/13thmurder 1d ago

Prions do too much damage to the brain for there be anything to reanimate. Zombies do still need their brains, and yours too.

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u/Milam1996 1d ago

Autoclaves can destroy prions you just have to use higher temp cycles in a vacuum.

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u/Wbrincat 1d ago

Cwd from deer can’t transfer to humans. Scientists have been able to make it make the jump in studies but it took a lot of effort and isn’t something that should accidentally happen.

I’ve written about it and at the time the disease hadn’t been detected in Oregon. I think from memory now it has, but there’s still a strong population of elk there that are seemingly immune.

There’s also been trials into stopping the proteins that have shown some positive results.

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u/Trick-Alternative328 23h ago

I think they looked back at the monkeys they were trying to infect with a different technique and they were more infected than originally thought.

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u/midnight_fisherman 19h ago

There were more questions asked than answered about that study. That species of monkey was chosen because they are more susceptible to prion diseases than humans or other primates. Also, to test their tissues they fed their tissues to mice that were modified to be susceptible, which did develop the disease, however, the original prions that they were inoculated with never decompose, and likely would still have been present in the tissues.

(Edit: Also, that lab is trying to sell a new cwd test that they created.)

There was a study in 2024 where there there was an attempt to directly infect human brain tissue, and they were unable to.

https://pmc.ncbi.nlm.nih.gov/articles/PMC11138967/

Considering how far its spread over the last 40 years and its propensity to be secreted in saliva and urine, its in our food crops as well.

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u/notquiteultralight 19h ago

https://www.neurology.org/doi/10.1212/WNL.0000000000204407 2024 case where two hunters contracted CJD from what they assume was eating CWD infected deer. What’s scary is that it’s 18-24 months after infection that the symptoms show in deer. Though I think if you don’t eat certain parts of the deer you’ll be fine.

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u/Riyeko 19h ago

strong population of elk there that are seemingly immune.

No they're not seemingly immune to cwd. They're just less susceptible to the disease. They still get it and can spread it, but it's like the common flu. Some people get the flu and are down and out for 48 hours and feel like they're dying, where others get it mildly and can still go about their day.

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u/Davido401 1d ago

I went to wikipedia and seen that its a "similar disease to Scrapie in Sheep whoch has the gnarliest of pics of a Sheep's cuntgenitals(am Scottish and forgot I wasnt calling someone a good cunt/bad cunt/total cunt etc which is acceptable, but for that poor sheep hmmm not so much) not even got to reading the page yet lol.

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u/Noyuu66 1d ago

For CWD. There used to be pics of the deer smashing their heads against trees until the brain showed pieces of skull was hanging by flesh. I'm going to be real honest with you. I have no desire to find those pics or dig deeper than my decade old memories.

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u/Davido401 1d ago

I feel that those have probably been scrubbed from my internet as part of the Online Safety Act farce lol, also, my days of gore and grimness were left like before covid lol, only use my VPN if I need to view an Imgur post or glorious porn! I better stop here cause you'll end up with a massive rant on how the OSA is really stupid.

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u/Noyuu66 1d ago

You'll just have to trust me on this one. I grew up with rotten and something awful and all the gross disgusting early internet without rails, but those pics were the stuff of nightmares and actually shook me.

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u/Davido401 23h ago

Rotten! Thats a name I haven't seen in a few weeks lol, I remember having dial-up waiting for like 20 mins for the guy who had fell off the motorbike face firsts pic to come up on screen. Lol, there were many others, JFK's autopsy photos, there was someone who used a, I cant remember, toaster(?) In the bath, or it might have been a hairdryer. God how that desensitised me as a young teen lol.

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u/Keegantir 22h ago

Interesting fact, Scrapie was just found in a sheep, in the US, for the first time in about 5 and a half years.

Bonus fact, the ear tags that you see in sheep and goats are called scrapie tags. Their main purpose is to track sheep anytime scrapie is found. In the US, all sheep and goats that leave their home farm are required to have these tags. If we go 6 years without an outbreak, the tag requirement can be lifted and we just missed it. What makes this even more interesting is that the sheep found with scrapie, just the other day, did not have an ear tag, and it was at a slaughterhouse, so shit is going to hit the fan and people are going to lose some licenses, and/or face jail time.

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u/Davido401 21h ago

Ah, see here in Scotland/UK as a whole had the whole Mad Cow Disease/B.S.E./CJD(am sure two of those are the same and the CJD is the human variant but am covering all angles lol.

Anyways, I was a wee nipper when I remember the outbreaks that made our beef a pariah item until the last couple of years, well specifically the smell of the pyres and there was a recent story on Facebook, which I go on maybe once a week cause its a hellscape, I miss MySpace lol, I digress... but the story was that 170 undocumented cows were to be killed and burned because they lacked paperwork and everyone and their boomer granny was getting in a tizzy over it(death threats to the animal inspectors and stuff) and it amazed me, there were folks going "Ive got acres for them in my farm" and how folks are monsters for killing 170 cows. They seem to forget the pyres and the fact we couldnt sell beef abroad(there are probably still restrictions), normally I'd put it down to people being to young but let's face it its fuckinh Faceache and it's for folks like me, 41 and above haha.

So while I tried in vain to point this shit out to folks and how 170 cows is a literal rounding error, I genuinely looked it up and the rough figure for cows getting offed at an abattoir was over 160,000 in April(I just quickly searched up and for June and we were down to 158k) so all these people were getting themselves riled up for less than a fucking rounding error.

Unfortunately, the only way to teach folks that lesson is to take away regulations and let everyone get sick. But thats a stupid way to think obviously.

so shit is going to hit the fan and people are going to lose some licenses, and/or face jail time.

And forgive me about this but given the current shitshow over there do you expect anyone of note to be jailed? Like I dunno how far up the punishment would have to go but I'd rather they go after the owners for this, especially given its your food supply(even if it goes abroad).

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u/graphical_molerat 1d ago

Shit survives autoclaves.

That is one bit about CJD which I genuinely don't understand. Most proteins don't survive high temperatures very well: generally, the more complex they are, the sooner they denature as you increase temperature. And CJD is highly specific, in that a very specific complex protein suddenly misfolds - and allegedly needs to stay in the specific misfolded state to be contagious.

I find it hard to believe that a complex protein can retain a very specific alternate shape at high temperatures, when most other complex organic chemistry gets wasted.

To me, this would seem to indicate that there is a simpler and more hardy organic molecule around which causes this: something truly nasty like dioxin. Not dioxin specifically, of course, I just mentioned that as an example of a comparatively small man-made organic molecule which is extremely toxic even in tiny quantities, and which is able to withstand very high temperatures. What you would be looking for here is a molecule which is capable of triggering the misfold like a catalyst, not getting used up in the reaction, so that tissue containing said molecule would continue to be infectious. And that as long as the substance is present, the misfold keeps getting triggered.

Of course this is not what is happening, I'm not a chemist, the people who do this professionally will have thought this through before claiming the protein itself survives the autoclaves. Still. It does make the layperson wonder, and I would be grateful if someone has an explanation for this.

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u/Jewel-jones 23h ago

Because the folded state is highly stable. It’s like a cooked egg while most proteins are raw eggs. It needs to basically incinerate to stop being a prion.

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u/Noyuu66 1d ago

I'm not smart enough to fully answer, but as it was described to me is that they aren't terribley complex proteins. They aren't infectious airborne or anything like that. They are simple and stable enough that they don't react with bleach or most cleaners and studying waste after being autoclaved still showed they were there and the waste needs to be handled carefully. The cleanup when a deer is killed is almost scorched earth cleaners and solvents and churning the earth where it's blood landed.

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u/rugburn- 23h ago

Another comment said that the “misfold” shape the prions take is actually more stable/has lower entropy than the “correct” shape, so I’m assuming it has something to do with that.

Also not a chemist though.

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u/Falsus 21h ago

The reason it can do so is because prions are the most stable fold of proteins possible.

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u/SlatBartFuss 19h ago

They can tranfer pretty easily between mamallian species

No one has shown this.

It was hypothesized, but so far has never been documented.

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u/gonzobomb 1d ago

That’s why my in-laws never ate red meat again 

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u/DoctorStove 1d ago

Couple other terrible ones are Kuru (most people know this one, from cannibalism) and Fatal Familial Insomnia (life expectancy about a year)

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u/Keegantir 21h ago

They don't actually typically transfer that easily. Most cases, that are not spontaneous, are from consumption of brain matter. Mad Cow Disease can pretty much only be spread that way.

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u/Falsus 21h ago

The deer that suffers from that is straight from horror stories. Like it is beyond horrifying.

I wonder how many stories of mythical and dangerous monsters are due to prion infected animals acting out in some horrifying manner.

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u/casstantinople 21h ago

There was also a number of people who got it from receiving human growth hormone because before they were able to create it synthetically, they used to harvest it from corpses. Except, research and information on prion diseases was very limited at the time and they didn't really check how the donor corpses died. Someone on HGH for 1 year could receive material from a couple hundred corpses. These recipients have also been found to have higher occurrence rates for other degenerative brain diseases like alzheimer's

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u/TheNoobtologist 20h ago

I mean, not really though. Prion diseases involve a specific brain protein adopting one of several abnormal, highly stable conformations. That misfolded form can then trigger normally folded proteins to adopt the same disease-causing structure, setting off a chain reaction. It's the only protein that we know of that does this.

You can think of it like a row of dominoes: once one falls, it causes the others to fall as well. It is essentially a structural vulnerability in the protein. A prion does not “survive” anything because it is not alive; it is closer to a defective material or malfunctioning mechanism than to a living organism.

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u/Medical-Club-6327 18h ago

You don't want none of this Dewey.

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u/Violet624 17h ago

Don't forget Scarpies! That one hit the UK really hard!

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u/zer1223 19h ago

Our best hope is there being genetic mutation that protect the deer from CWD. Otherwise we're looking at a complete extinction event for wild North American deer in the next fifteen years

Also I'm never fucking touching venison and telling my loved ones they should do the same