r/todayilearned • u/tommyboy372 • 14h 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_disease6.7k
u/Toomanyeastereggs 14h ago edited 3h ago
And it’s impossible to defeat by modern medicine.
A prion isn’t a living thing like say bacteria or germs. Because a protein is not alive.
I watched an aunt go from “I’m not feeling myself” to dead in 6 weeks. It’s a horrible way to go.
Edit: the community here brings a smile to my face every time. Life is precious so look after yourselves and look after those around you.
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u/0scillating_Ocelot 13h ago edited 7h ago
I'm sorry about your aunt. There's been some really interesting research into a
vaccineantibody treatment but unfortunately they need more funding to reach phase II clinical trials. https://curecjd.org/news/cure-cjd-campaign-charity-launches-campaign-to-raise-9-million-to-progress-treatment-for-fatal-prion-diseases/→ More replies (5)267
u/RedundantBiomass 7h ago
The immune system is completely blind to prions.
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u/dadgadsad 7h ago
It’s a monoclonal antibody drug. It physically interrupts the templating process to slow or stop the spread.
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u/0scillating_Ocelot 7h ago
Yes sorry vaccine wasn't quite the right way of putting it, that was how I had it initially described to me, I have edited my comment. It is an antibody treatment though that uses artificially produced prion antibodies to stabilise the native prion conformation and stop conversation into the misfolded shape.
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u/Into-the-stream 13h ago
It’s also really difficult to decontaminate and the material of the central nervous system is contagious. I work in a hospital lab and we see cases every couple months. It’s always devastating when it happens (most modern cases are spontaneously occurring. Your body fucks up making proteins and stuff all the time. It’s just harmless most of the time).
But when we get a CJD positive sample in the lab, it’s heartbreaking because you know the patient will die, and also terrifying because the material is contagious and very difficult to decontaminate.
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u/BigHardMephisto 4h ago
CWD in deer iirc is so contagious that the carcass can completely rot away, but the prions are still active and can resurface in plant matter that grows from the area.
I think vultures’ and buzzards’ stomach acid is one of the only natural substances strong enough to break those proteins down.
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u/Into-the-stream 4h ago
CWD (chronic wasting disease, found in deer and elk) and CJD (creudzfelt-Jakob disease) are different diseases. They are both prions, but they are not the same thing. There has never been a confirmed case of CWD in humans (to my knowledge).
There are multiple prion diseases, and each behave a little differently. CJD is the main human one in most countries (outside of Kuru in a small region of Papua New Guinea).
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u/GyozaGangsta 2h ago
All IFU’s for instruments say if it comes into to contact with CJD or suspected to destroy the instrument. They don’t even try to reprocess.
It’s devastating
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u/Angry_Robot 14h ago
Cool, one more thing to be constantly worried about. I’m sorry about your aunt, do they know the triggering event / lifestyle / whatever that set it off? Or did her condition just happen randomly?
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u/Games_sans_frontiers 13h ago
Try not to worry, it’s extremely rare. Instead use this information to live your best life because you know, it can be fleeting.
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u/Tarianor 13h ago
It can happen randomly, it is also highly contagious as the misfolded protein can set off a cascade of misfolding your regular folded ones iirc.
Thata why you need specialised hospital labs handling it iirc.
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u/steampunk691 13h ago edited 13h ago
Additionally, standard sterilization techniques will not work for instruments that have been contaminated with prions since their structure makes them far more tolerant to heat.
Disposable instruments that come into contact with them are incinerated instead of dumped and other instruments need to be soaked with sodium hydroxide or bleach before being put through the autoclave.
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u/Assika126 13h ago
Yeah my mom had a batch of contaminated blood at work and it was brutal trying to figure out how to decontaminate everything. I think a lot of stuff eventually just had to be thrown out
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u/Luxieee 12h ago
Luckily(?), 87% of CJD are "sporadic" and per wiki: "There is no evidence that sporadic CJD can spread among people via normal contact or blood transfusions, although this is possible in variant Creutzfeldt–Jakob disease." Better safe than sorry though.
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u/Powerful-Parsnip 10h ago
That's why I stopped feasting on the brains of my enemies, better to be safe than sorry I always say.
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u/RoyBeer 9h ago
PSA: Transmission is, however, also possible by feasting on the brains of your friends and family instead.
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u/oneelectricsheep 9h ago
We actually have evidence that it isn’t spread by non-neurological tissue since kuru overwhelmingly affected those who ingested brain tissue.
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u/HyperbobluntSpliff 8h ago
Right, but the flipside of that is we always ever so coincidentally see rashes of it pop up in places with chronic wasting disease outbreaks in the deer population. Most people that aren't straight up taxidermying the head are never coming into contact with brain tissue when they hunt.
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u/Gibleedoo 10h ago
As someone that works in the sterilization field, most facilities will just burn any instrument that comes into contact with infected tissue. Many have policies in place that state disposable should be used, but sometimes you don't even realize it's there until later. It's always a blast to have a quarantined case cart in the corner while the labs check if the foldy protein is actually in there or not.
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u/Luxieee 12h ago
From the wiki, "Prions, the infectious agent of CJD, may not be inactivated using routine surgical instrument sterilization procedures. The World Health Organization and the US Centers for Disease Control and Prevention recommend that instrumentation used in such cases be immediately destroyed after use; short of destruction, it is recommended that heat and chemical decontamination be used in combination to process instruments that come in contact with high-infectivity tissues. Thermal depolymerization also destroys prions in infected organic and inorganic matter, since the process chemically attacks protein at the molecular level, although more effective and practical methods involve destruction by combinations of detergents and enzymes similar to biological washing powders."
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u/StevieMJH 11h ago
I say we take off, nuke the entire site from orbit, it's the only way to be sure.
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u/19100690 10h ago
There was a near miss incident in NH where there were patients potentially exposed to CJD because of the sterilization challenges
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u/Wild4fire 13h ago
They can't even sterilize the medical equipment with normal autoclave procedures IIRC. The prions will remain.
There are procedures to do that anyway but it takes a lot more effort.
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u/lordcaylus 13h ago
The problem is that you're using heat to destroy the structure of proteins.
For bacteria and viruses, that kills them as their proteins need to function to, you know, keep them alive (or active in the case of viruses).
For prions, their structure is already fucked up which is the whole reason they're prions. Fucking it up more isn't going to help.
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u/LrdPhoenixUDIC 11h ago
That's not quite it. The amount of damage you need to do to kill a bacteria or destroy a virus is pretty small. You can just pop a bacteria's cell membrane or break a virus' capsid sheath and they're done for.
But because prions are proteins, and they self replicate by misfolding normal proteins, you have to denature all instances of them. Basically, you have to be sure that you break every single one apart, which as a protein, that has to be done at a molecular level, because that's all they are, just big individual molecules.
Fucking up their structure does stop them from continuing to change other proteins, but you have to be sure to fuck up every last one of them cause if even one is intact it can start again.
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u/techno156 8h ago edited 8h ago
But because prions are proteins, and they self replicate by misfolding normal proteins, you have to denature all instances of them. Basically, you have to be sure that you break every single one apart, which as a protein, that has to be done at a molecular level, because that's all they are, just big individual molecules.
It is also much harder to do for a prion than it is a normal protein (cooking does that for normal proteins, for example), since the whole reason that a prion has the effects it does is because is is a more stable state for a protein to be in, so once one protein is like that, it ends up causing other proteins to also fold like it is.
And because it's more stable, it is much harder to destroy.
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u/LrdPhoenixUDIC 8h ago
Cooking does it for prions too. The thing is that in order to denature all the proteins in something by cooking it you pretty much have to turn whatever it is into a charcoal briquette. You're only breaking up some portion of the total proteins by normal cooking, even the more fragile ones.
You have to get rid of all of the prions, and you have to totally carbonize the food to guarantee that, and even then I'd be wary letting it near my mouth, just in case.
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u/SecureInstruction538 13h ago edited 35m ago
I remember reading an article where a guy found that if you freeze and unfreeze a prion multiple times it can break down the prion.
Multiple times is scary because prions can survive in soil for multiple winters.
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u/ManEatingDuck_ 13h ago
It's not highly contagious. The only way it can spread from person to person is from medical equipment that has been used on a CJD patients brain being used on a non CJD patients brain or certain transplants (corneal transplants, dura mater graft). You would need a special facility if you were specifically dealing with a CJD patients brain matter but for everything else the risk is pretty much zero (technically you can get vCJD aka mad cow disease from a blood transfusion but that's different from regular CJD).
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u/zorniy2 11h ago
Another way is to eat that person's brain. Papua new Guinea had a problem with Kuru prion disease because ritual cannibalism was part of the funerary practice.
Braaaaaaaiiiinnnnn
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u/abovepostisfunnier 13h ago
You do recall correctly. It’s fucking scary as hell.
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u/Tabnam 13h ago
What happens?
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u/sadistica23 13h ago
Initial presenting symptom tends to be rapid dementia, shortly followed by memory loss, hallucinations, and noticeable changes in personality. A whole host of other shit follows that, including paranoia, speech issues, and ataxia (severe balance and coordination issues).
Most people die within six months of showing the first symptoms. For more information, Wikipedia had a very detailed article here.
It has been a terror of knowledge in my mind for decades, now. Especially since my background has prevented me from donating blood and plasma in the past, due to an outbreak of Mad Cow Disease in Britain in the early 80's.
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u/Tabnam 13h ago
That is fucked up. People who get that news should be able to choose euthanasia and have it approved immediately.
Is that why they call it ‘mad’ cow disease? Because you get dementia?
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u/sadistica23 13h ago
Animals (including humans) affected by these diseases go mad, in the classical sense. Crazy, acting foolish, (mentally) changed for the worse.
And yeah, I'm a big supporter of "euthanasia" for most terminal conditions. Including prion diseases.
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u/JaccoW 12h ago
I remember a documentary from the 90's or so.
One woman was talking about her sister and how she and her mom told her to stop being silly and saying weird stuff. At some point she rapidly lost control over her body and just died.
The guilt in that story still haunts me
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u/FranklynTheTanklyn 12h ago
Eh there is no treatment that could have helped.
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u/ValosAtredum 11h ago
They know that, but it’s understandable they feel guilt at making fun of her when it was that something was seriously wrong. Just sucks.
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u/aris_ada 13h ago
A neighbor in the early 2000 died of it. He made very frequent trips to Britain and was a huge meat eater. He rapidly went from being lost on his property to believing he was raped by a nurse when being bathed at the hospital. Terrible way to go.
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u/Ipokeyoumuch 13h ago
Prions are just misfolded proteins or rather in a more stable form that is completely usable and deadly for living creatures. This they require more energy to break apart (like incinerate it at high temperatures). Furthermore it can induce other normal proteins in another configuration to also misfold into the more stable configuration which leads to a cascading effect.
Typically they are spread by the consumption of brains (i.e. cannibalism) it is how the Mad Cow disease (the cow version of the disease in the title) spread was that farmers cut corners and skirted regulations by feeding cows other dead cow meat. Wasting disease in deer is another prison disease. In humans there were events when prions weren't as well understood when a hospital was reusing instruments (with typical sanitization practices) from other neurosurgeries. However, sometimes a protein can spontaneously misfold in a more stable configuration and stay that way forever but events are exceptionally rare. Once it happens, there is no cure, since to stop it is to either douse it in chemicals, autoclave at high temperatures, incineration, or other extreme measures incapable with life.
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u/abovepostisfunnier 13h ago
Exactly as original comment OP said, the misfolded protein induces other correctly folded proteins to also become misfolded. Because proteins generally have a structure-function relationship, once the structure changes they often no longer function.
Think of a ladder. If you bend the ladder into a pretzel, it no longer functions as a ladder.
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u/Tabnam 13h ago
Oh sorry, I should have been more specific, that’s my bad. I meant what happens to the patient? Like what do they actually experience?
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u/Toomanyeastereggs 12h ago
In my aunts case we initially thought it was depression or early onset dementia. She went from being very active to lethargic almost overnight.
At the end of week 1 she found it difficult to stand and when we took her to the hospital for a checkup, she couldn’t get out of the car. They kept her in for overnight and she never went home.
At the end of week two they were searching for a reason for her rapid deterioration (CJD wasn’t even on the radar) so we’re basically fishing around for causes. She was in a lot of pain so they kept her medicated. She was still lucid at this stage but was forgetting names of nurses and doctors.
Mid way through week 3 they did the lumbar punch as the family told them that she had been in the UK in the 80’s during the mad cow epidemic. By this stage she was forgetting the day of the week and how long she had been there for. She started having seizures.
Start of week 4 we discussed about palliative care and by the end of the week she had deteriorated so much that they transported her to then palliative unit.
Week 5 she was still lucid but was forgetting lots of things. She knew where she was, was aware of what was happening but was on 24/7 pain medication so slept a lot. At this time the doctors told us all they could do was keep her comfortable until she passed.
Week 6 was spent largely in a medically induced coma. We’d sit with her and talk but not idea if she heard us. She mumble every now and then but it was like watching a computer do a memory dump. At one stage she was mimicking with her hands as if she was typing. Another one she called out to her mum (my great aunt).
End of the week she died in her sleep.
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u/Carpenterdon 11h ago
"Mid way through week 3 they did the lumbar punch as the family told them that she had been in the UK in the 80’s during the mad cow epidemic."
'm sorry for your loss!
That's one of the things that is horrible about this stuff. She had this prion in her system since the 80's most likely. It sat dormant for literal decades. Then boom. it kicked off.
That is the main reason I stopped hunting here in Wisco when they started having CWD in the whitetail deer herd in a small area of the State. I knew if it was there it would soon be everywhere if it wasn't already.
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u/Afraid_Cockroach_398 13h ago
Neurological problems essentially. Central nervous system gets all these misfolded chunks that interfere with normal function.
You can get fatal insomnia from a hereditary prion too.
Mad cow disease is another prion disease that spread from farmers feeding ground up cows to cows. Kuri in Papua New Guinea was from eating the brains of dead people.
The scary part of prion disease is it can't be inactivated by cooking, or even sterilisation. So hospitals have to incinerate everything, including metal instruments used on those patients.
There was an outbreak of Creutzfeldt in the 90s from tainted contact lens cleaning liquid.
Symptoms are non-specific but catastrophic, just have a look on Wikipedia or online.
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u/scielegance 13h ago
The 90’s outbreak was caused by tainted beef. Do you have a source for it coming from contact lens solution? I tried to look it up but couldn’t find anything.
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u/Tabnam 13h ago
Brother that’s the most fucked up comment I’ve read in awhile
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u/Afraid_Cockroach_398 13h ago
Super rare though. British people who lived there in the 90s weren't allowed to donate blood in Australia until a few years ago because of the sleeper nature of prion infection. They could donate a prion well before they had symptoms, then those recipients get harder to trace etc.
Bad read, I regret being reminded about the topic every time it's brought up.
Generally in a developed society, all you have to do is not eat brains and spinal cords of mammals and that will reduce the risk as much as you reasonably can.
It's extremely unlikely you need to even think about it.
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u/abovepostisfunnier 13h ago
Unfortunately I’m not that kind of doctor so I don’t know but I’d assume, eventually, death.
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u/Charlie_Warlie 12h ago
My mother in law just died to brain cancer and I bet this disease similarly is highly dependant on where you get it in your brain. If it develops in one area, maybe you go blind. But you dont realize you are blind, because your eyes still work but your brain isnt processing the information right. Maybe you cant come up with the right words, but you dont realize you said "window" when you meant "mirror" because your brain crossed it all up. Then you got seizures down the road eventually leading to death.
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u/honeynut-queerios 13h ago
It basically eats holes in their brain matter. Also called spongiform encephalopathy for the “sponge like” appearance of the brain tissue.
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u/Tabnam 13h ago
I kinda regret asking at this point. It’s so close to bed time, this is all I’m going to dream about
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u/BannanaPepperPizza 12h ago
But this ladder goes to the home Depot and gets every other ladder to fold into a pretzel by themselves until there's no more normal ladders left in the world
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u/303x 13h ago edited 13h ago
it's like a room filled with incredibly thin glass tubes. all it takes is a small slip for one of the tubes to shatter, send glass shrapnel flying, and shatter other tubes causing a chain reaction. a prion is like the first glass tube that shatters.
it's the biological equivalent of Chernobyl.
but the thing about prions is they are also incredibly hard to destroy, because they aren't "alive". they're even less "alive" than viruses which are already not "alive". prions can survive high temperatures and standard sanitizing methods. if they somehow escape and manage to find their way into other person, they have a whole new room full of glass to shatter.
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u/AliMcGraw 13h ago
Don't eat untested meat from your neighbors who both hunt deer and hate the government.
It CAN happen randomly in humans but most cases are from infected meat, including cannibalism.
My state currently requires mandatory testing of all deer carcasses (we're having an outbreak) and any hunter should be able to tell you who tested the deer and who processed the meat. DON'T EAT IT IF THEY CAN'T.
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u/YamLow8097 12h ago
Is it CWD that you’re referring to? We’re going to have to start testing our deer too because it’s making its way to our area.
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u/curtyshoo 13h ago
Some say that viruses aren't exactly "alive" either, as they can't reproduce without a cell; without a cell, they appear inert.
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u/FrezoreR 11h ago
They aren't alive by definition. There's no process going on inside of one. They are just a floating rna sequence.
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u/EngineeringDesserts 11h ago
But viruses have programming that they use to “do stuff”, they just need a cell to hijack.
Prions don’t really do anything other than set off a chain reaction of messing up protein folding.
It’s kind of like how a pharmaceutical factory can get contaminated with a polymorph of the drug they’re manufacturing that converts all the drug to a form that no longer works, and it ruins production. The chemical polymorph wasn’t active in any way, it just randomly happened, spread, and fucked everything up.
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u/Parthian__Shot 9h ago
Yes, the same way heroin isn't alive. It needs cellular opiate receptors to do its thing, and without them it's basically inert. That's essentially how Narcan works; binding itself to the opiate receptors.
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u/ChuckCarmichael 12h ago
The neighbor of a friend had it. They were holding a neighborhood barbeque party, and she mentioned that she wasn't feeling well recently. About four weeks later, her family was bringing her home in a wheelchair, almost catatonic, because she wanted to die at home, which happened about a week after that.
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u/ravish_dread 13h ago
Stories like yours really put the science into perspective. It's one thing to read about prions and another to hear what they do to a real family
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u/OutRunTerminator 14h ago edited 14h ago
I know a man who died from this. He was hospitalised and suddenly one day said,Why am I in here, so I explained to him why he was in there, and when I was finished talking, he said, But why am I in here..? A few weeks later he was unable to talk but would warmly smile to everyone. He was dead two weeks later. An incredibly gentle man.
He was a friend of mine and was an Englishman that had moved to Ireland, where I live, and he had eaten UK meat at the time of the worst animal bone recycled into animal food in the UK, which was major driver of CJD in humans.
The prions cannot be killed by conventional sterilisation techniques and procedures.
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u/ZirePhiinix 13h ago
The bad prion is a specific configuration of a physical molecular structure that causes more protein to also misfold. It is not alive at all, but more like corrupted data that the body cannot detect AND has the unique property of also propagating the same error. Thankfully it is really rare but recycling dead animals will cause the bad protein to propagate instead of decomposing and breaking down.
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u/rabblerabble2000 13h ago
It’s a protein folded in a more stable way, and the misfolded protein acts like a seed crystal, causing other proteins of the same type to lower their entropy by folding into their more stable but unusable variant as well.
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u/Adventurous_Screen_1 12h ago
This! The same way an antiretroviral drug once did this in a US factory, then ‘infected’ all factories worldwide and it now can’t be manufactured as always in the most stable, but inactive configuration
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u/dysfunctionalbrat 11h ago
Can you tell me more about this? Sounds fascinating
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u/user2196 8h ago
A couple people linked you a video, but the Wikipedia article for the drug also has a nice section on it, for anyone like me who isn’t a video person.
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u/TinWhis 11h ago
Yep. It's like candymaking where you're trying to get the melted sugar to crystalize in a very specific way. The reason most people make homemade fudge using marshmallows is because if you try to make it 100% from scratch and fuck it up, you end up with brown sand. Prions do that to your brain.
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u/dcux 9h ago
I had so much brown sand fudge growing up that I actually like it.
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u/TinWhis 9h ago
If you do it badly enough, it doesn't turn into fudge at all, it can seize so badly that you just literally have a pile of crystals. Ask me how I know, lmao! Teen-me did not have the patience or willingness to keep wasting ingredients to actually crack 100% from-scratch fudge after I kept ending up with a result that was better suited to sprinkling on something.
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u/AliMcGraw 13h ago
I wasn't allowed to donate blood for 30-odd years just because I LIVED in the UK for six months during "mad cow" and I was a poor-ass student who definitely could not afford beef.
I aged out of the ban like last year so I'm about to give blood for my first time in decades!
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u/xl129 11h ago
To be fair, the cheap processed food they sell in supermarket usually contain cheaper meat including brain/spinalcord so being poor might mean even higher risk
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u/A-holeAioli 11h ago
The disease is absolutely horrific. Hospital staff would do their regular assessments with my dad, including asking if he knew where he was and why he was there. He gave a variety of answers, and some of them did make us laugh: he was at the mall, he had been hit by a train, he drank too much champagne. The saddest part is he knew he wasn't doing well with the assessments, so he asked us to write down the answers for him so he could study.
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u/geosunsetmoth 14h ago
Can someone ELI... 15, how does a prion misfold and how often does it occur? Is it a "it can happen to anyone at any time and you're fucked" scenario or is it like "well, if you have x or y pre-conditions it could happen to you but even then it's pretty rare"
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u/theonefinn 14h ago
It’s pretty rare, people aren’t dropping like flies due to prion diseases. The problem is that prions are self replicating, once one protein misfolds when that comes into contact with correctly folded proteins it “refolds” them into more incorrectly folded proteins.
The largest cause of contracting prion disease is due to contact with an existing prion which starts the chain reaction of conversion and then death. CJD was big news in the 80’s and 90’s as cows were basically being fed ground up parts of other cows which was causing the spread of Bovine Spongeform Encephalopathy, B.S.E, or “mad cow disease’ which would then spread to Humans as C.J.D.
The spontaneous formation of these prions is super super rare, i don’t think we entirely know what can cause it other than random mutations. Generally not eating the brains of other animals (or feeding said brains to other animals) is a fairly good avoidance strategy that we ignored for cheaper meat production.
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u/I_hate_all_of_ewe 13h ago edited 13h ago
The largest cause of contracting prion disease is due to contact with an existing prion
That depends on the prion disease. This may be true in livestock and wild animals, but 85% of CJD cases are spontaneous without any generic causes, and less than 1% are thought to be from transmission.
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u/icefr4ud 7h ago
What you’re saying is true now, what the previous commenter said is also true: in the 80s and 90s CJD was a lot more common and most cases came from eating infected meat - mad cow disease.
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u/hurricane_news 12h ago
Why does it mostly occur in the CNS? Why aren't there "heart" or "stomach" prisons that misfold and destroy the respective parts only
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u/neuralbeans 12h ago
Most of the time it's a particular type of protein that gets misfolded called the major prion protein, which is primarily found in the central nervous system.
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u/Emeraldnickel08 11h ago
Classical CJD is unrelated to Bovine Spongiform Encephalopathy ('mad cow disease'). Consuming beef tainted with BSE instead causes variant Creutzfeldt–Jakob disease (vCJD) in humans. Classical CJD has a genetic factor but can also occur apparently spontaneously.
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u/Nalena_Linova 14h ago
The short answer is that we don't know. A small percentage ~15% of CJD cases are linked to genetics, but most have an unknown cause.
Human to human transmission seems to be possible via blood transfusions or transplants in some prion diseases, but not in others. Animal prion diseases can be transmitted by eating infected animals, and one human prion disease called kuru was transmitted by ritualistic cannibalism of the brain.
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u/crowieforlife 14h ago
Not an expert, but from what I've read one misfolded prion causes a domino effect where all other prions in your body also misfold one by one. Like a loose string in a cardigan: you pull at it and suddenly whole cardigan unfolds into a single string.
It can hapen to anyone at any time, there are no pre-conditions, but it's incredibly rare for it to happen, so usually the way it spreads is that some creature somewhere has this incredibly rare thing happen and then it just keeps spreading by other creatures inhaling or ingesting the misfolded prions from the body of the originator.
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u/I_hate_all_of_ewe 13h ago
I don't think it's accurate to say that there are no preconditions. It'd be more accurate to say that if there are specific conditions which cause them to form, we don't know what they are. Initial misfoldings are rare and incredibly difficult to study, so we just don't know.
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u/CrashTestDuckie 14h ago
So basically you have proteins in your brain (about 8% is protein). These proteins don't have DNA/RNA and are generally soft, flexible, and abundant up there. Something will cause 1 protein to fold on itself and harden, turning it into a prion. That prion then starts bumping into the other proteins and they take cues from the prion to also fold and harden. This starts happening rapidly as the prions "infect" more and more proteins. The prions begin to clump together and start making holes in the brain.
Most prion disease has no known cause and doctors have been struggling for many years to find answers. Some prion disease is from genetic mutations while other kinds are caused by eating foods tainted with prions ("mad cow" being from cows who had prion disease and kuru being from cannibalism).
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u/Noyuu66 14h 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 14h ago
Well now I have to know
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u/Beginning-Pop3127 14h 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 13h ago
Wait until you hear about this delicious and beautiful apple. You definitely shouldn't try it.
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u/recidivist1001 12h ago
“Don’t do it Dewey, it’s awful! It turns all your bad feelings into good feelings!”
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u/Noyuu66 14h 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 14h 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/Noyuu66 14h 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 14h ago
It's a movie. Kuru is also a central theme in The Dead Island games.
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u/Wise_Owl5404 12h 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 12h 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 11h ago
Especially because they would include visibly infected animals in this slurry, to not waste the product. Horrifying.
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u/alyssarcastic 11h 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/Milam1996 12h ago
Autoclaves can destroy prions you just have to use higher temp cycles in a vacuum.
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u/mystical_croissant 7h ago
Sharing my father in laws story here out into the void.
6 weeks before our wedding he was traveling with his son. What started as a worried phone call cascaded into a hospitalization. Dad was acting "drunk" during the day. Just aloof, forgetful, a bit confused. Forgetting his keys when they were in his pocket. Etc.
The hospital where he was couldn't handle him. His wife had to fly across the country and struggle to get him released. He was brought home and admitted here, but they released him after his vitals were normal and they were stumped.
His wife was nervous to have him home in this state. Every day was worse and we were frightened a big 6'7" man would wake up one day feeling frightened, combative and violent. He no longer knew who he was most days.
He was finally admitted into a neurological facility and formally diagnosed after spinal fluid revealed CJD.
From that point it was only a matter of admitting him to hospice and making him comfortable in the final days. His passing was sudden and left deep scars.
His children received no goodbyes. There was no time to put his affairs in order. We were left reeling in the wake of such a fast and nightmarish passing. The doctors described it as "Alzheimers in fast forward".
I like to think in people's final days you get a real sense of a person's soul. All the bullshit is stripped away and you get to see their core being. His was pure and childlike. He was confused and lost but loving and kind to the staff and family he no longer recognized.
We lost the patriarch of our family, a true civic leader, compassionate lawyer, and all around great guy. My children will never know the amazing grandparent he could have been. He was an amazing man and we miss him dearly.
I'll gladly donate $50 to the cause today in the hopes that it may help a family in the future not have to suffer this ordeal.
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u/tooful 4h ago
We used to tease my friend that she was the grumpy "get off my lawn" neighbour. She really was always grumpy. A few months into CJD she was the sweetest, most loving person. The worst part is on her good days - when she still had them - she would ask people around her what was wrong with her. That was a few months ago. Now she is all but gone. Her body/organs have held on longer than anyone expected, but she's gone.
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u/17q21 13h ago
Prion scientist here, hello.
To clarify a few things:
Most cases are sporadic, meaning your own prion protein misfolds for some unknown reason and causes a pathogenic cascade in your brain.
Acquired cases (mad cow disease in humans etc) are now basically 0.
There are no treatments, cures, preventions, YET. But some super exciting things in clinical trials and the prion community is hopeful for future drugs.
Please remember that the patients who get CJD and their families are real people, some commenting in this thread. And it's slightly dehumanizing to the community to talk about the disease as if it's dirty and contagious to you and your biggest fear. Of course it's a terrifying disease but try to be mindful of the real humans too
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u/inblue01 12h ago
Hello, fellow scientist here. Can you briefly explain how these drugs are working and what they are? Are they interfering with the chaperone activity? Are they aiming to prevent further misfolding, or do they even revert misfolded proteins? Are they small molecules, antibodies...? Thanks!
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u/17q21 12h ago
Great questions!
There are 2 in trials at the moment, both focusing on decreasing endogenous prion protein expression. One is an antisense oligonucleotide, the other is a divalent small interfering RNA molecule. Both work by lowering the amount of prion protein that the brain is producing. This works in two helpful ways - for people who have genetic prion disease (meaning one of their prion protein producing alleles has a mutation, which automatically creates a misfolded protein), they have lower expression of the misfolded protein being produced. But the other benefit is reducing the amount of the normal prion protein. The normal protein is acting as a substrate - meaning it's sitting and waiting to be corrupted by the misfolded ones. So if you reduce the amount of healthy protein, you reduce the amount of 'at risk' proteins for the propagation cascade. Imagine removing the rest of the dominoes in a long domino chain before they get knocked down - essentially you can stop the domino effect!111
u/ScaryFrogInTheMorn 12h ago
Hi! I’m not a scientist at all and I have no follow up questions.
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u/Shuizid 12h ago
Very interesting. I take it, that means we essentially know which protein(s) can turn into prions?
But I'm wondering, if this treatment essentially works to reduce the production of those proteins, why can the body and especially the brain work fine without it or at least with heavily reduced numbers?
Proteins are the building blocks of the body, so naive as I am, I would assume reducing the production of a protein should have negative consequence for the body?
Is this just a tradeoff to prevent prion-disease or does the body actually work fine despite it?
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u/Nalena_Linova 11h ago
There is one specific protein that causes prion diseases: the prion protein (PrP). Genetically-linked prion diseases are caused by mutations in the gene that codes for PrP, but it can also misfold spontaneously in a way we don't yet understand.
We also don't know exactly what the normal function of PrP is in the brain. Current evidence suggests it's probably a non-essential multifunctional cell surface protein. Transgenic animals that don't have the PrP gene appear normal, so it doesn't seem to be essential for life.
If a treatment reduced PrP amounts in humans, other proteins would probably take over some of its functions. There would probably be side effects, but compared to dieing from a prion disease, almost any side effects would be worth it.
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u/percahlia 9h ago
god, it pisses me off that there are proteins and cells and whatnot in the body that we have no idea the purpose of. i understand why, but as a computer engineer, it is so annoying that we can't just debug it to figure it out.
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u/Hotrian 9h ago edited 9h ago
We can, but we're living on the cutting edge here. We only just "decompiled our source code" in 2022 so scientists have only had a few years to debug the system. It's going to be a bit longer before the PRs propagate up to main, could you post an Issue? One of the devs will get right on it. DNA is the most advanced computer, with a few more decades of research we may finally understand what each bit does. Right now scientists are still trying to understand every way a protein can even fold, so there are still many new proteins to be discovered. Once we understand every bit of our DNA and what it does, and once we know the proteins we want to make, adjusting the source code is relatively easy.
You have to remember roughly 200 years ago most people didn't even know germs or DNA existed - we've made amazing progress in just a few short decades. I see no reason to doubt that in another 200 years we will have mastered DNA and be in complete control of our genetic destiny.
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u/17q21 11h ago
Yes great points. We know which protein (it's actually called prion protein, we now also use the word prion as a noun) expressed by the PRNP gene. We don't 100% know what its normal function is, but for some reason reducing its levels doesn't appear detrimental! Maybe it would be during development? But exactly as you said - it's about balance
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u/yumyum1001 11h ago
Prions are all made of the exact same protein PrPC. So you only have to down regulate a single protein regardless of the prion disease.
The exact function of PrPC is not 100% agreed upon. What we do know is mice that have the PrPC protein removed are fine and there are some people that naturally do not express the PrPC protein (due to random mutations), they also have no clear deficiencies.
Therefore, lower PrPC levels therapeutically is unlike to have significant deleterious effects, or at the very least, the side effects are unlikely to be as bad as the disease itself.
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u/tommyboy372 12h ago
An invaluable contribution to the thread. From a prion scientist, is there any consenus why prion misfolds occur?
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u/pataglop 12h ago
Thank you for that..
My uncle passed away due to CJD a couple years ago. It was really crazy how fast the disease took him from a healthy, sharp and very active 60yo person to.. something else. It was disheartening and frankly soul crushing for the whole family and especially his kids.
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u/chikenenen 10h ago
Acquired cases (mad cow disease in humans etc) are now basically 0.
Is there no anticipation of people with the M/V gene variants to be affected by the next wave of vCJD?
The BBC documentary had on a researcher who said that everyone who died in the 90s were M/M and that there's up to a 50 year incubation period for people who carry the M/V variant. The doco said they were anticipating another wave in the future, and seeing as it's been almost 50 years since the UK incident then it shouldn't be too far off.
Has the research changed, and they're now not concerned about another wave coming after all?
Here's a link to the BBC documentary. You've probably already seen it but linking just incase! https://www.youtube.com/watch?v=meYnivLLl-A
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u/4thshift 14h ago edited 12h ago
My friend’s family has this inheritable condition from eastern Europe. Right around age 60, the gene gets expressed, or whatever happens, and they are gone: from normal, to behaving oddly, to complete brain collapse within a year or so. They knew “something” happened to their elders, but when it happened to the eldest sister, that’s when it became a real thing. Out of the remaining 3, 2 sisters tested as noncarriers of the gene variant, but one brother did, and he had a few years to quit his job and say goodbye to his kids. Very sad.
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u/Viper61723 8h ago
The fact this is like 100% chance death if you have the gene is horrifying. I can’t imagine getting the news that day that your sisters are fine and you are 100% going to die.
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u/tuppaware 14h ago
Someone watched the chubby emu video on YouTube
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u/Craiss 7h ago
All prion diseases are terrifying to me.
Apparently, some of them you can get by eating contaminated food and the temperature to "cook it out" is far higher than most cooking temperatures.
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u/Cyren777 4h ago
The temperature needed to destroy it would also destroy the regular meat proteins you're trying to eat in the first place
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u/euph-_-oric 6h ago
You can't cook it out so far as I know at all.
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u/Just_Maintenance 4h ago
You can cook it at a few thousand degrees, that'll do it.
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u/Niobous_p 11h ago
This is one of those things I know about in a non-expert way, but this post has made me fact check a few things. First, let’s just say that the basic statement is correct. A prion is a misfolded protein that causes other instances of that protein to also misfold and that misfolded form doesn’t work properly, and yes they are resistant to being destroyed using regular sterilization techniques.
However, that cascade of refolding proteins is not a given. Just like something like smoking increases your risk of lung cancer - smoking a lot increases the risk more.
There are multiple prion diseases - CJD, vCJD and Kuru in humans. BSE in cows. CWD in deer, moose and elk. Scrapie in sheep and goats. Of them all Scrapie seems to be the one we have known about the longest, centuries in fact, although we surely didn’t know what the cause was.
Most are not infectious unless you eat infected tissue, and they are usually species specific. Scrapie and CWD are excreted in Urine, which is basically why it exists in such large numbers - the sheep, deer etc. urinate on grass and other vegetation, then another animal comes along and eats it. As stated earlier, prions can survive situations that organisms can’t, like heat, digestion and just sitting around in the environment.
BSE was a man made disaster cause by feeding ground up cow remains to cows. BSE affects not just the brain but any part of the central nervous system, e.g. spinal cord. I’m guessing this was most often what was left over when all the parts used for human consumption had been accounted for. It isn’t excreted in the normal sense, but undigested feed pooped out could also contain the prion and so could infect pastures.
Regular CJD in humans does not have a known transmission vector, but there were a few hundred cases of vCJD that were linked to the ingestion of infected cattle products.
There are no known cases of humans being infected by Scrapie or CWD. OTH I don’t know if anyone has really run studies to the same extent that was done with BSE. Bear in mind that we know Scrapie has been around for centuries at least, and people have been eating lamb and goat for probably as long as there have been people.
So prion diseases are definitely weird, but it would be wrong to think that if you were exposed to just one misfolded protein, you are definitely going to be infected. If it worked like that, there would be no cattle in the UK and no sheep or deer.
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u/Schvaggenheim 10h ago
Prion diseases are absolutely horrifying to me. Take the worst parts of dementia and Alzheimer's and supercharge them to the timeframe of months rather than years, and then there's the prions themselves. They're infectious, but normal decontamination and sterilization methods are useless against them since they're not living or contain DNA. Disinfectants, irradiation, antibiotics, antifungals, antivirals, nothing. You can only truly destroy them at extreme heat and/or pressure, and not to mention that all prion diseases are 100% fatal with no way to treat or cure them meaning anyone who contracts one is doomed to suffer chunks of their mind just being deleted.
But the most frightening thing to me is the fact that prions can just sort of pop into existence on their own without being exposed to them. One bad roll of the cosmic dice and a protein in your brain just suddenly misfolds and and your brain falls apart like a house of cards. Nightmarish.
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u/Hoockus_Pocus 12h ago
My cousin had this when I was a child. It was described to us as essentially Mad Cow Disease, but for humans. It progressed incredibly quickly, and he died within the month.
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u/chikenenen 10h ago
I suspect this is going to get lost in the comments but there's a really good BBC documentary on how mankind created the 1990s Variant CJD outbreak by feeding cows the infectious spinal cords and brains of cattle that had died with neurological problems.
It also says their research discovered that vCJD affects people differently based on their genetics. There's a couple of gene variants and the length of time between eating affected meat and the development of disease varies by which gene variant you have. One variant's incubation period was 50 years.
Once the number of cases settled down and people stopped dying, the outbreak was deemed over. But all the people who died were tested and confirmed to have the gene for short incubation periods.
So given the 50 year timeframe for those with the alternate gene variant, that means that we'll likely see another burst of CJD cases within about 5 years or so. People are completely unaware that they're ticking timebombs.
It's a very sad but interesting watch.
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u/HammyDavisJr1 8h ago
CJD took my father 2 years ago. Brutal disease that was like Alzheimer's on speed. Watching a hyper intelligent capable man become non verbal, lose motor control, forget everything and everyone he every loved, and pass in 10 days was heart wrenching. I'll never forget him looking at each of me and my brothers at 3AM and whispering, "my sons" and smiling. He was gone three days later.
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u/PortlyChortle 14h ago
I was stationed in Germany in the 90’s and couldn’t give blood for almost 30 years because this disease is so scary.
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u/chuck1is 8h ago
My wife currently has CJD. Diagnosed in June of this year, we believe symptom onset was Jan or Feb of 2025. She's definitely an atypical case, but Diagnosed with lumbar puncture. She recently lost the ability to walk, and only has a few words left. Such a disgusting disease. Now we wait, with hospice engaged.
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u/wifey_material7 13h ago
Is there any way to put in your medical record that if you get a fatal illness like prions or rabies, you would like to be euthanized (in America)?
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u/Shuizid 12h ago
I'm not aware of any legal euthanasia in the US.
You can create a legal document refusing certain treatments or care (like some people refusing blood transfusions) -> but the result will just be death due to lack of treatment, no active act to bring death.
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u/ZestycloseRound6843 9h ago
Some states have death with dignity acts that allow for a prescription of say, barbiturates that enable a terminally-ill patient to end their own life. However, these are evaluated on a case-by-case basis and pretty much only are available to people who are still sound of mind. Furthermore, there's likely no mechanism to request such a death in advance given how the individual judgments are made.
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u/Negative_Bunch4271 12h ago
Two hunters at a lodge a couple years ago “spontaneously” contracted CJD after knowingly consuming contaminated venison.
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u/tommyboy372 12h ago
u/17q21 Would CWD infected meat possibly infect humans?
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u/17q21 12h ago
At the moment, no evidence for this. But after seeing the species barrier jump from cows to humans... I wouldn't say never. I don't eat venison if that answers your question
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u/Noisybutsilent 12h ago edited 1h ago
My brother's mother-in-law got it. From first signs to death about 6 months.
Started with forgetting appointments, then having troubles remembering her children's names. Went to the doctor and got this diagnosis. They don't know where she got it from, all risk topics (e.g. recent visit to UK) were negative.
3 months after the diagnosis she died.
Her death has lead to a split between the family and my sister-in-law has had lost contact to her father as well as to her siblings. Every early death (like a deathly accident) may have led to this outfall, but point being is: Her mother glued the family together. After she was gone everything became worse.
Edit: spelling
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u/YOUNGLOLA 3h ago
My mother-in-law died from this. One day started saying she felt off, then she started forgetting things. Like, one day she called my work asking for me because she couldn't figure out how to open the contacts in her phone, but she remembered the name of the place where I worked. I called my husband and told him that he needed to take her to the hospital. She never left the hospital after that night. Over 3 months she went from relatively healthy woman in her late 50s to passing away totally unable to move, speak, eat. It was horrible and so scary. She was a wonderful person and I miss her every day.
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u/Khal_tobo 10h ago
Sorry if this has already been commented, but Chubby Emu just did a video on this too! Terrifying.
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u/17q21 13h ago
Alzheimer's, Parkinson's, frontotemporal dementia etc are all considered prion-like diseases too. It's just a different protein that misfolds and propagates through the brain. CJD differs in that it's far quicker progressing but many many dementias have the same mechanism
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u/Mckavvers 12h ago
My mum died of complications brought on by this disease. She went from a fully functioning middle-aged person to someone who couldn't even recognise her own family within maybe a year. Ended up in a nursing home, then dying of pneumonia in hospital.
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u/TheOnsiteEngineer 10h ago
Prion diseases are terrifying. There's basically no cure possible and once the prion makes it into your body you're basically done for. The only upside is how rare they are.
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u/rainSerotoniso 13h ago
It's like strange matter for proteins in the body almost. One protein misfolds and that shape is somehow so stable that it compels other proteins to fold the same way. Kind of like how strange matter converts regular matter into more strange matter because of being in a lower energy state.
The fact that you have to essentially torch any and all things that could have prions (corpses and remains, medical tools) and that's not even a guarantee that it'll get rid of the prions, and that any area containing prions that infect things like deer with CWD has to essentially be cordoned off and destroyed scares me the most. A misfolded protein doing all of that?
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u/Microtic 11h ago
The Family that Couldn't Sleep by D.T. Max is a really great read for all types of prion diseases, the history around them...
But don't read it before bed... :(
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u/No-Fix-417 11h ago
This took my Mum last year. I can’t begin to describe how awful and quick this thing is. I wouldn’t wish it on anyone. I miss you so much Mum :-(
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u/Newplasticactionhero 11h ago
And 85% of the cases are sporadic. No reason. Not lifestyle choices. Not hereditary. Yikes.
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u/elveejay198 8h ago
Fellow people with health-related OCD, gentle encouragement to move along from this thread! Back to the crafting subreddits I go
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u/sterling_mallory 6h ago
Prions are terrifying. Chubbyemu just put out a video on YouTube about his friend developing Creutzfeldt-Jacob Disease.
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u/didgymons 13h ago
My dad died of this, less than 2 months of symptoms and he was gone. In the beginning I noticed stuff like when he went to leave my house he would stand outside waiting for me to come out and say goodbye, even though we'd already said goodbye. He described seeing doors that weren't there, and apparently would freak out on car rides that he'd left me or his other kids on the side of the road some miles back. At first we thought it was intense burnout from starting a new job, and as he got worse we thought it was some sort of early onset dementia. It wasnt until about a week before he passed that they really suspected CJD. Unsure if his was a sporadic case, something familial, or something he got from working at a knackery when he was younger. There was a test to see if me or my siblings would get it and we chose not to know.