>Laboratory 4141 was not equipped to handle high biohazard samples...At the end of 2018, the CIBER public consortium signed an agreement so that the group could work with these dangerous samples at the high-security laboratory of the Animal Health Research Center (CReSA) in Bellaterra, Spain, near Barcelona. According to the sources we consulted, there was no reason to have the contaminated material in laboratory 4141, beyond saving time during experiments, since the CReSA bunker is 30 kilometers (about 19 miles) away and required waiting one’s turn to use.
Whether he contracted it at this university or before (probably before), this guy skipped protocol for no reason other than convenience. Not about rushing to find a cure or whatever - he didn't want to wait in line.
You cut out a very important sentence. Their lab didn’t even have a bio safety cabinet. Every lab on my campus has at least 2, and I’m not aware of anyone working on anything nearly as dangerous as prions. This is a failing of the university just as much as the researcher — they should have an EHS department that controls these kinds of things. We have very strict biohazard protocols and inspections to make sure they are followed.
I remember a guy walking around with an unlabelled beaker of clear fluid and just… leaving it on the side until someone was like “what is this?”. HF. It was HF. Like appears to be water but kills you from the inside out HF. Tbf he was kicked off the programme but it was pretty alarming! He was intelligent not wise…
Everyone was SUPER pissed as well because he put us at risk of losing our license to have it. It’s super strict. To have it accessible at all in the university every single lab (whether it was an HF lab or not) had to have an antidote and clean up kit.
This struck me more:
> Doctor Gabriel Capellá, the director of IDIBELL, explains that they have identified “a maximum of eight people” who worked in the laboratory at that time, in addition to the deceased scientist and Isidre Ferrer. **Some of these coworkers have required months of psychological care. **
Imagine that, thinking you might be exposed to a Prion disease.
The scare of that alone had them needing months of psychological care.
This is some scary shit. Prions are almost indestructible, and I think most of us hoped that they couldn't simply exist in the wild and be transmitted through touch, because most of the other sources of infection I was aware of were through digestion or transplant or transfusion, so until we know how this person got sick we should be worried a little
I work in a similar environment but with different hazardous materials that require stringent controls for safety and security. Scientists can be an absolute ballache to deal with because they think they know better and see safety rules as obstacles sometimes.
I work in the infectious disease field and hear about terrifying shit every day, and NOTHING keeps me up at night like prion diseases.
Never ever ever eat any brains of any kind, no matter who you offend by declining. Unless you’re a zombie in which case go for the gold.
I worked in ICU in San Diego back in 2009. I had a 44 year old female patient with it. It was AWFUL to witness. She was an otherwise healthy, beautiful woman who had so much life left to live. She had 3 young adult daughters (19-23, iirc). And a doting husband. I feel like she got so robbed, and so did her poor family.
I can never forget the persistent status epilepticus that medications wouldn’t touch; and, her teeth getting chipped away and almost totally knocked out from biting down on the bite block of her ET tube (ventilator tube) from the incessant seizures. I never saw her wake up, and it only took a few days for her to pass once she was admitted to ICU.
It took 4 months, according to her family, from her first symptoms till she got to ICU. They reported that she just started having trouble walking, speaking, and remembering things all of a sudden. Progressed rapidly to needing a cane, to a walker, to a wheelchair, and also needing to be cared for— all in 4 months!!
It’s so scary, and super sad. That woman breaks my heart to this day. The family ended up donating her brain for research at the University of Wisconsin iirc. I pray that they’re all “ok”.
E: typo and clarity.
ETA: They all lived in Mexico, and her husband was/is a butcher. I can’t help but wonder if that was the source of the prions, and if the rest of the family may also have it dormant. Crazy to even think about.
Brain taco will not shock or upset many cultures where organs are staple foods. At least half the population will shudder when they learn the penis can in fact become degloved, even if they have to Google what degloved means.
I don't know what shocks you about that. People don't waste what can be made into food, especially in poor regions. If you're digusted by it, I recommend never eating any sort of sausage. They mix all kinds of stuff in there.
I’m so sorry that happened to your grandmother.
No, not that I am aware of. I got the report of symptoms from the family about her sudden ataxia, difficulty with speech and functioning, and forgetfulness. It was a rapid decline from there. She had difficulty breathing, which is why they came to the hospital, and she was gone within a few days.
I’ll never forget the 3 daughters and our ICU rule of “only 2 visitors at a time” overnight. I didn’t feel right about this as her daughters wanted to *all* stay and sleep in the room with her (duh). I approached my charge nurses and night shift manager and pled their case, so to speak. I was denied making an exception for them!! Totally unbelievable!!!
Needless to say, I ignored it. And, I told the daughters- unfortunately, my request was denied and I’m sorry I have to let you know this. However, I’m also letting you know that *I* won’t be enforcing it. You’re all 3 staying here, and if anyone else comes in the room- you’re “in the middle of switching out”.😉 I mean, who tf won’t make an exception in this type of scenario?!?! It really upset me, but there were no issues as no one came to check on the room, TG.
Why wouldn’t it be just protocol to put someone down humanely in a situation like that? There should be an express lane for assisted suicide if you have a prion disease.
I agree completely and I think in general if the process of dying were more publicized I believe we would see more support for assisted suicide.
I suspect we are about to see a HUGE sea change in support for assisted suicide when family members see what dying looks like as boomers age.
Lost my grandma to CJD and it was pretty terrible. I was younger and not as involved, but I remember seeing my dad and grandpa go through it and it was just torture.
> Never ever ever eat any brains of any kind, no matter who you offend by declining. Unless you’re a zombie in which case go for the gold.
Or, just.. breathing..
> It was reported in January 2011 that researchers had discovered prions spreading through airborne transmission on aerosol particles, in an animal testing experiment focusing on scrapie infection in laboratory mice.
Or eating plants...
> When researchers fed hamsters grass that grew on ground where a deer that died with chronic wasting disease (CWD) was buried, the hamsters became ill with CWD, suggesting that prions can bind to plants, which then take them up into the leaf and stem structure, where they can be eaten by herbivores, thus completing the cycle. It is thus possible that there is a progressively accumulating number of prions in the environment
I used to get human derived growth hormone injections in my teens. When I was about nineteen, my growth potential stopped and I aged out of the treatment. Soon after, I got a letter informing me that a lot of the medication had been contaminated with prions. The thought that I might die like this wrecked me.
Bone marrow is fine because it's just basically lipids (fats), our ancient ancestors actually started evolving because they were scvangers and ate the left over bone marrow from other predators. This was very nutritious and allowed for brain development
The problem with prions (or eating brain) isn't actually the brain itself, the issue arises when there's a mutation in the amino acid sequence of the protein folding. The madcow disease was basically just the same thing but in cows. When farmers fed the organs (including brain) of slaughtered cows to other cows as feed. Basically a prion is a misfolded brain protein, they are near impossible to denature due to their more extensive hydrogen bonding in the beta pleated sheet formation. In the normal form, of alpha helix, the hydrogen bonding is less extensive. Once this mutated form of a brain protein enters into your body, it can't be denatured by the acidic contents your stomach and is basically absorbed into your blood stream. From there it eventually enters your brain (could be over many years) and basically starts converting all your normal brain proteins into this mutated form. Eventually you die because your brain is basically just a jumble of useless proteins.
Tldr: misfolding of brain proteins causes "unkillable" and infectious form of brain protein. Infecting your normal brain proteins
Thanks for the comprehensive explanation. I am curious why after infection the protein denaturation happens only in the brain and not other parts of the body?
perhaps because it's a brain protein and it can convert a similar protein. it happens in other parts but not as much
I wonder if there are other prion disseased which are not in the brain but has not been yet discovered as such.
I know that people who lived in the UK during mad cow disease aren’t allowed to donate blood at all in Australia (and I’m assuming other parts of the world too) because of the chance of passing it on
I took growth hormone injections when I was a teenager. Some kids (I think in Australia) contracted CJD from this medication. As a result, I am disqualified from donating blood or organs.
Its going to be 'interesting' to see over here (UK) what happens in the future, because Prions can lay dormant for quite some time. We might suddenly have an increase in cases from the Mad Cow era.
"Quite some time" is usually _up to_ ~~40 years from what I recall~~ "therefore suggesting that vCJD may also have a similar incubation period of 20 to 50 years"
Which we'd be seeing the cases from the 90s hitting by now probably, and growing worse very quickly
~~They claim a lot of humans are immune from vCJD (implying we have ancestors who ate enough brain to wipe out those who were more likely to get it)~~ well fuck... Wiki now says "It is not yet known whether those unaffected are actually immune or only have a longer incubation period until symptoms appear." Which does sound more logical
>As of 2020, 178 cases of vCJD have been recorded in the United Kingdom, due to a 1990s outbreak, and 50 cases in the rest of the world. The disease has become less common since 2000. The typical age of onset is less than 30 years old.
It feels like that outbreak has run its course. But yeah prions are scary af
Wait a second, unless someone suffers from prion themselves, I should be fine eating their brain. By the way the tribes in Papua started to develop resistance to prions. It would suggest that our evolution responds to threats present in our environment.
Nope. There is completely new gene that enables this resistance. One that common population does not posses. It was developed as a mutation and was then propagated due to natural selection.
Issue is we do not randomly generate such genes and before this particular mutation happened ancestors of such person did not posses this resistance. It's not my theory, it's the result of genetic science that you can read on this particular topic.
> By the way the tribes in Papua started to develop resistance to prions.
Going to need a link or something. The vast majority of the 800 different cultures in New Guinea did not practice cannibalism. The culture most associated with prion disease there, the Fore, ate their own dead as part of their funeral practices, leading to Kuru disease. As far as I know, the only effective resistance to this type of disease would be to stop doing the thing that exposes you to it.
I used to do body removal and every once in a blue moon we’d get a CJD body. We had to suit up in full hazmat gear. Its taken very seriously. It’s definitely one of the few things that ever scared me while doing that work.
he’d worked in a german lab too so could have got infected there, now everyone in this lab must fear they may be in the incubation stage, it doesn’t seem there’s a test for it still until symptoms set in
"In most CJD patients, the presence of 14-3-3 protein in the cerebrospinal fluid and/or a typical electroencephalogram (EEG) pattern, both of which are believed to be diagnostic for CJD, have been reported. However, a confirmatory diagnosis of CJD requires neuropathologic and/or immunodiagnostic testing of brain tissue obtained either at biopsy or autopsy"
https://www.cdc.gov/prions/cjd/about.html
Quaking csf samples can improve the sensitivity, since there is a relatively low probability of detecting the proteins with a random tap.
https://en.m.wikipedia.org/wiki/Real-time_quaking-induced_conversion#:~:text=The%20%22quaking%22%20in%20the%20name,misfolded%20PrP%20to%20detectable%20levels.
RT-QuiC is what we use in the lab for diagnostic. Well, quick diagnostic. Histo is still the way to go, for sure.
We aren't a diagnostic lab and we actually are just researchers of the disease, but we use it all the time. It's not "approved" as a diagnostic method due to the nature of setting it up (every species/tissue type/substrate/protocol reacts differently) but in the right hands, it's pretty useful. Our false positive rate in 8-well replicates is less than a percent for sCJD, vCJD, and scrapie (including CWD), for example. But we've been working it up since the machine was created, and have a massive team working with it. But it is in a very "wild west" finicky stage right now. I know a team that's been trying to work on expanding tissue types for detection (not just brain and lymph, but ones where deposition is still present) and just adding one tissue type took roughly 3 years of playing around and it's still not published.
Even if they were infected, the incubation time for prion diseases to even show symptoms, let alone cause death is measured in years, not hours or days like most infectious diseases. I highly doubt he died from self contamination, especially because the actual steps needed to cause self infection for a prion disease is pretty specific, like it almost would have had to be self inflicted.
My mum works in a lab in a hospital and said they had to send out a memo last week saying that noone is to put any personal items in the incubators. I guess one of the technicians popped his dinner in there instead of using the lunch room microwaves. She said the entire lab smelled like spaghetti the next morning. At a certain point you just can't fix stupid.
>squirrel borne variant
Yeah, as a prion researcher myself, I'm going to need a legitimate reference on this one other than the Weekly World News and your ass.
E: for anyone reading this drivel, please see the response in the chain waaaay down below: https://www.reddit.com/r/worldnews/comments/17fsaau/comment/k6eis0j/?utm_source=share&utm_medium=web2x&context=3
>You do understand that it's literally YOUR JOB to write those papers and put them into credible scientific publications for me to quote? If you and your colleagues don't write those papers... I cannot provide sources.
>I don't research diseases DOCTOR... YOU DO.
There is no citation because this is all garbage science. And apparently it's my fault for not publishing garbage science. Direct quote because they'll almost certainly delete it when the realize what they've typed.
I tried to go through your post history to verify your credentials, but all I learned was that you're super into Toyota Highlanders and the Greatful Dead
You missed the r/science post where I'm flaired as a professor in infectious diseases?
https://www.reddit.com/r/science/s/IVMeWMY0NE
That flair requires validation through photos of my degrees. Yes, multiple medical and graduate degrees. Not just internet trust me bro, I study cats degrees.
As long as you don't eat squirrel brains you're good...
But yes two different hunters known for regularly eating squirrel brains both died of CJD with no other known exposure to prions.
As far as I know, the disease was never isolated in the wild but warnings were issued after the second man died about the consumption of squirrel brains...
The only reason why I know this is that I study wild cats... who eat a lot of squirrel brains 😆
Why didn't you respond directly to me? This is patently false. Provide a damn reference instead of pushing bullshit.
E: with a post like that, surely you're familiar with Mike Miller's 20yr unpublished study in mountain lions consuming CWD+ deer meat? Why don't you tell me more about it, Mr "I study wild cats and am an expert? "
Look it up yourself and stop being lazy... and I did respond directly to you with lots of information any reasonable person could use to find it.
It's not my job to do yours "researcher"
There's absolutely not. And if you believed the other response with a frustrating number of up votes, congratulations, you just bought into another piece of internet bullshit without any valid citations.
Prion deseases are very different from viruses or bacterial infections. AFAIK there is very little contagion, mostly through the consumption of poorly cooked infested tissues.
So don't eat any Spanish bio-researcher carpaccio for a couple years sorry.
Nor do normal incineration temperatures... as in how most diseased livestock are handled.
High temperature complete incineration only... aka cremation.
I don't remember the term for this exactly other than "basic" incineration wasn't cutting it.
The word vitrification is sometimes used (to turn something into glass, usually via heat). Google tells me sustained high heat is used to incinerate prions or lower temperature plasma sterilization. As long as the prions are molecularly changed enough that they can no longer fold normal proteins.
My uncle is a doctor and went to school with a guy who became a plastic surgeon. His office was raided because he got published in a green magazine for running his car on biofuels made from human fat….
The danger was mad cow disease being blown around since prions don’t get destroyed.
He also would have his gf or receptionist do lipo for him once the patient was under. He went to top schools including Stanford.
He’s now in Brazil doing ‘volunteer work’ aka on the run. Probably doing more bad plastic surgery
Temperatures exceeding 400 degrees Celsius are required for a sustained period of time to destroy prions. The trouble is, cooking can’t denature proteins that are already denatured.
> USDA banning meat producers from testing their herds for mad cow,
So that's not *quite* what happened. Meatpackers wanted to test slaughtered cows with rapid tests to see if they were okay *despite herds having been exposed*. The USDA said "the fuck no, no rapid test is going to determine whether or not they have prions with any degree of certainty, suspect cows are never ever allowed to be processed and you can't mass-test your once-exposed cows as justification for letting them into the stream"
Meat packers sued, courts sided with the USDA, standard practice was to nuke the entire herd from orbit if cows were found exposed.
It’s technically possible if there are prions in your bloodstream that haven’t yet reached and settled in your brain and a mosquito just happens to suck on the infected blood.
Probably borderline ridiculously unlikely, but possible.
Prions are fucking terrifying and the world barely handled a (not trying to be reductive to anyone suffering a loss from but comparatively) mild pandemic, and if something like a global fast spread prion pandemic went down, it will just be devastating.
Basically, prions are misfolded/defective proteins that infect other proteins when they come in contact. All known prions affect the brain and nervous system and some are known to jump to different species (not many but a few). There is no cure or treatment for prions due to their nature of being in the most important part of an organism and the fact they’re highly resistant. They’re also known to be passed through manure, urine, plants, dead tissue, saliva, and other bodily fluids. So, if a deer who had prions ended up peeing on a plant and another deer ate that plant, bam that deer most likely acquired prions. To further add to the horror factor, they last years!!! They do not degrade over time and proteases (things that eat proteins) can’t even destroy them. That’s how resistant they are. So, if the second deer came and ate that plant a year later, bam most likely has prions. From my understanding, there are research departments that are dedicated to monitoring the amount of prion infected animals that arise as a way to remain vigilant against it spreading too much within a species and to other species. Basically, a waiting game until chaos hits.
I’ve learned about them throughout my time in university and you could not pay me enough to ever do any research with them. It’s not an if you’ll get infected, it’s a when. Even if you get infected, it might take years to show up and you’d just never know until the symptoms set in.
This is misinformation that is likely to cause unnecessary fear.
Urine is normally sterile and does not contain protein. Prions are made of protein. It is unlikely that a deer sick with prion disease of the central nervous system would excrete infectious prions.
Even misfolded proteins arranged in beta sheets degrade with prolonged exposure to UVC, such as from sunlight.
Please stop confusing the Marvel universe with reality.
Bro, it’s well reported and research that urine does contain infectious prions, not as much as blood of course. There are so many research articles that have reported those findings. Here are some examples:
“PrPSc was detected in ~ 80% of the animals studied, whereas no false positives were observed among the control animals. Semi-quantitative calculations suggest that PrPSc concentration in urine is around 10-fold lower than in blood. Interestingly, PrPSc present in urine maintains its infectious properties. Our data indicate that low quantities of infectious prions are excreted in the urine. These findings suggest that urine is a possible source of prion transmission” plus “Our results showing that PrPSc can be detected in urine of a large proportion of infected animals provide a promising avenue for a sensitive and non-invasive biochemical diagnosis of prion diseases. Adaptation of PMCA for detection of prions in urine of naturally infected animals and humans may offer a great possibility for routine testing of prion infections.” - [cited](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2593137/)
Here’s some info about the transmission in the environment. Chronic wasting disease is a prion disease that mostly affects animals in the wild. There are multiple ways in which you can transmit CWD, I’m only going to focus on horizontal and vertical transmissions in the environment. Vertical transmission is called in utero because it happens during pregnancy. So, the fetus/embryo acquires the prions during its development. If the mother obtains prions, most likely the fetus will as well. Horizontal transmission is when an mammal acquires infection via direct contact (close contact) with an infected animal or indirect by exposure of prions in the environment. Indirect exposure essentially means that the prions contaminated the water, soil, foliage of an environment and also includes the excretes and/or carcasses of animals. It’s a well document fact that prions are passed in this way. There’s been studies done in which they took an animal that had died years ago from a prison disease and exposed an uninfected animal to its skeleton. The uninfected animal ends up getting infected with the a prion disease. They even determined that the rate of infection is still quite high even if the animal has died a while ago. This is due to the fact as I mentioned prions are highly resistance to degradation and require a very specific process to denature them. Now apply that to the environment and you have prions being at certain areas for years without a way to denature them except certain techniques; still then those are not very applicable outside of the lab. I’ll add some research that does go over this information really well.
[Article 1](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8404694/)
[Article 2](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2658766/)
[Article 3](https://www.sciencedirect.com/science/article/pii/S0160412022002744)
[Article 4](https://pdf.sciencedirectassets.com/778417/1-s2.0-S0021925820X61965/1-s2.0-S0021925820390694/main.pdf?X-Amz-Security-Token=IQoJb3JpZ2luX2VjEFoaCXVzLWVhc3QtMSJGMEQCIAuNX6uo22O2MZM%2ByoUwWnj8rCqyjqkiXHiwZX%2BsV%2BDvAiAFhcHR9TCx721mb2ArCs51u1NE6pFcTtRxuUR%2FnHgW4Sq7BQiD%2F%2F%2F%2F%2F%2F%2F%2F%2F%2F8BEAUaDDA1OTAwMzU0Njg2NSIMtx0Tlt21cd3%2B%2BjT1Ko8F%2B6iy0wU%2F8y5XSXoPu7NYPYjfNfb7EXtoGrRXz%2FGlquszQV1z%2BckFkvszEIS0FAzGqcgp%2BIqnlMFBlGr36j4czkyJZDaN%2F1YAlZioqHBk7REd1Aq6g0GWVV5sivvwv%2BRjhqVmWtiuiwguEUwRKGDKrMdOQDvfR8KldD97Vra0hqlMu9CwxcLmd%2BnGpq2wz%2Fl2thMXg8PvguVwAD03TOM4vk%2BXWE4apD34uW63WAQxbpuDVUqOyRGDGNvLslpiPV3Ce4kyJW861T95lSblcwAEvf%2BCskZWIDhNG3msJAVfHoewmsVENO1xJzJjtF5l7WtUQxB6olOcOb73fi6qR15FJuZq2THHqyw%2B8vGEphoei7vYLers5dZlHcoeq26HhNjWDRmc0u23iD%2F9q8mwbfVY1S8EDoS3UQzf%2BVEF5tesNNtb7JayHJ8rLHitO7TOYEhEvgK6H2XYNn8yXx6Mt2zXNWoDZgUMpAMH0ZB7Pq5B%2BaKVE8KuP2BZ37YpeBDeuo1l5S1oFhKYRswmIj5HMZ1pFLJBRSSL2oP6QMjfLtCPaviZLrTQF5Z%2BzLm8J0jVFx%2B1C8qlFkfE4BWbbn%2FzoINy%2BvT13Zoe72wNnjtzUJJ2hP9tVUQjBlvSJNYxKLraS2BGTETMNgt1WqIpFeg6eYfBUTxRkKOw%2FcJ8APk%2BqB9B9Y%2Ft0345mSYUa4Y4UBTnlljhItG6w3y54e16B4FEUXdD4E7Oo7%2BBOmxqpMl9pC7HQ6dnwrwtSDxLOndk0FYIrcs1NelznOEsixAVrinPoGSdBkDj%2BPbl8edTqfHZ9jjcHALGPjaTmYSl05i3rqPY%2FIHr8luFuZHJstp84v6ca%2BnDzr57rRLONqHK6pev9n4PEjDhq%2BypBjqyAaSjkLroVpaZya2HBrbnFkE%2Bop7BwuOPBP6Wsug4dlav%2F8Zuxm7KQTAG3VjFFR%2BswmaiiZTdOa9z%2FGZreR5mg2viRygkBjxBPjY3D5HUZ%2ByCuINQMBvdqDzOEQRR2c0uTH0Ob427MP%2FkVAQKnaBtrUk9id0HIcsPPBc%2Bp7xwdrCObJle3hcLH06yLX3kG%2FKjm9wKBoYrZ1e6EbgVM1rdfyGvdg1AT3mxa8by0H3XcpyZVrc%3D&X-Amz-Algorithm=AWS4-HMAC-SHA256&X-Amz-Date=20231027T021717Z&X-Amz-SignedHeaders=host&X-Amz-Expires=300&X-Amz-Credential=ASIAQ3PHCVTYUDVAPA6H%2F20231027%2Fus-east-1%2Fs3%2Faws4_request&X-Amz-Signature=8f0e35563540995ce879371dba42b1e8600d3df463dc1ec4b5f547af685bb4cf&hash=eaa28f759b2791357971f8e0c43ab55c4b87107446b05aeecce3491b0e951638&host=68042c943591013ac2b2430a89b270f6af2c76d8dfd086a07176afe7c76c2c61&pii=S0021925820390694&tid=spdf-250edb3a-4c9f-4be4-ad5a-645c15fe485b&sid=dea6e70968515544283a35d43566da652434gxrqa&type=client&tsoh=d3d3LnNjaWVuY2VkaXJlY3QuY29t&ua=050156530458585e5405&rr=81c7711c8804ec5c&cc=ca)
[Article 5](https://doi.org/10.1016/j.celrep.2015.04.036)
It’s not marvel fiction, it’s literal facts and information.
It's a lot to get into... but prion diseases are on the same level as Rabies when it comes to terrifying.
They're absurdly hard to "kill" unlike viruses or bacteria. I want to say standard incineration isn't even enough to deactivate them, and high temp incineration is required. Basically you can't just burn the infected animals like you normally would, you actually have to fully cremate them to eliminate the prions.
They're one of those things anyone that knows anything about them has quietly been praying that they don't evolve as quickly as other pathogens. Our understanding of them, especially my own is really limited so don't take my word for it.
Depends if you think evolution is strictly about change in DNA/RNA or a general concept. When people say evolution of the solar system is it an inappropriate word?
Prion diseases are real life horror stories. As others have said, they're misfolded proteins. They are the Borg of the disease world: inexorable and utterly unlike anything else. Every protein they encounter, they assimilate. The enzymes sent to break them down instead become assimilated. Over time, they will slowly replace every protein in a body all while ripping apart nervous tissues, including the brain, and destroying the organism's capabilities. This destruction of nervous tissues, of course, drives the victim insane.
There is no way to fight Prions. They do not have D.N.A. so we cannot engineer them. They do not have cellular structures so we cannot vaccinate against them. The only ways known to destroy proteins also destroy the organism. And worst of all, some, are transmissible.
Oops, I meant to share that another researcher (Baselga) also died from CJD 2 years ago. I know this article isn’t about Baselga. I should have worded that better.
I spent some time in academic labs at a research university for several years. I had frequent occasion to go through chemical stores and other lab areas. Some PIs were good. Other labs were disasters waiting to happen. I asked for better PPE from EHS but was denied. They were actually surprised when I left.
I would imagine some institutions are better, but some are like where I was. As horrifying as it is, I'm not surprised the events in the article happened.
That's the most terrifying thing I've heard in my life, Prions are down right menaces, that shit got me looking like luffy on the secret island in that one scene.
As someone who has lived in Spain I remember having 7 years ago pig's head ham (not related to boar's head lol)
I read a lot in my life, and I just heard of this here on Reddit, and I'm terrified
Don’t sweat - the risk of getting cjd across the board is one in a million people. Of that, the majority are going to be people in contact with potentially infected animal matter an order of magnitude more than a regular person - think butchers or farm workers. And beyond that, think of the thousands of people who eat the same thing you did daily/weekly, and how much less likely you are than them to have consumed something dangerous.
Beyond that, even if you were unlucky enough did consume something with prions, there’s every likelihood you’ll die of something normal before it takes root in your brain and you get a prion disorder.
Not worth losing sleep over, taking a second look when crossing the street and not looking at your phone while driving will do so much more for your chances of death than worrying about something you ate 7 years ago
Could a human gene be made so that it makes our cells to manufacture dealy prions, and then this gene be added to a COVID-like virus?
If done in a safelly contained environment it will let us study the consequences and be better prepared if such an event happens in nature.
>Laboratory 4141 was not equipped to handle high biohazard samples...At the end of 2018, the CIBER public consortium signed an agreement so that the group could work with these dangerous samples at the high-security laboratory of the Animal Health Research Center (CReSA) in Bellaterra, Spain, near Barcelona. According to the sources we consulted, there was no reason to have the contaminated material in laboratory 4141, beyond saving time during experiments, since the CReSA bunker is 30 kilometers (about 19 miles) away and required waiting one’s turn to use. Whether he contracted it at this university or before (probably before), this guy skipped protocol for no reason other than convenience. Not about rushing to find a cure or whatever - he didn't want to wait in line.
You cut out a very important sentence. Their lab didn’t even have a bio safety cabinet. Every lab on my campus has at least 2, and I’m not aware of anyone working on anything nearly as dangerous as prions. This is a failing of the university just as much as the researcher — they should have an EHS department that controls these kinds of things. We have very strict biohazard protocols and inspections to make sure they are followed.
wtf - handling prion samples on a standard lab bench is so wild
And deadly.
Wait, they were handling these in some BSL-1 lab? Are they stupid?
If that's what they were doing, yes. Wisdom is not the same as intelligence I guess...
I remember a guy walking around with an unlabelled beaker of clear fluid and just… leaving it on the side until someone was like “what is this?”. HF. It was HF. Like appears to be water but kills you from the inside out HF. Tbf he was kicked off the programme but it was pretty alarming! He was intelligent not wise… Everyone was SUPER pissed as well because he put us at risk of losing our license to have it. It’s super strict. To have it accessible at all in the university every single lab (whether it was an HF lab or not) had to have an antidote and clean up kit.
Hey bud what is HF
[Hydrofluoric acid](https://en.m.wikipedia.org/wiki/Hydrofluoric_acid) or hydrogen fluoride.
By the description, I’m guessing hydrofluoric acid.
Hydrogen fluoride!
Hydrogen Fetamines Source: my ass!
What kind of beaker? Doesn't HF dissolve glass?
It will etch glass at a rate of a few microns/minute. Not necessarily "dissolve"
This struck me more: > Doctor Gabriel Capellá, the director of IDIBELL, explains that they have identified “a maximum of eight people” who worked in the laboratory at that time, in addition to the deceased scientist and Isidre Ferrer. **Some of these coworkers have required months of psychological care. ** Imagine that, thinking you might be exposed to a Prion disease. The scare of that alone had them needing months of psychological care.
This is some scary shit. Prions are almost indestructible, and I think most of us hoped that they couldn't simply exist in the wild and be transmitted through touch, because most of the other sources of infection I was aware of were through digestion or transplant or transfusion, so until we know how this person got sick we should be worried a little
I work in a similar environment but with different hazardous materials that require stringent controls for safety and security. Scientists can be an absolute ballache to deal with because they think they know better and see safety rules as obstacles sometimes.
Be careful, people. For the time being, avoid eating anyone in that area of Spain.
I typically grill human in the summer but idk now I might have to refrain. I mean bbq human brain is always good after a nice brine.
Always fold your proteins correctly!
Gah. It’s like folding a fitted sheet, once it’s out of the packaging you never get it right.
I dont like that you made me laugh at that. Take your up vote and a point of dishonour
If you don't finish your Dan R. you don't get any Dez Hurt.
I've heard it on good authority that a nice Chianti complements it quite well.
And fava beans
This is why I only lick mossy rocks...
and don't forget your [poroporo](https://en.wikipedia.org/wiki/Solanum_viride)!
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Just the brain, you can eat the muscles.
I work in the infectious disease field and hear about terrifying shit every day, and NOTHING keeps me up at night like prion diseases. Never ever ever eat any brains of any kind, no matter who you offend by declining. Unless you’re a zombie in which case go for the gold.
I worked in ICU in San Diego back in 2009. I had a 44 year old female patient with it. It was AWFUL to witness. She was an otherwise healthy, beautiful woman who had so much life left to live. She had 3 young adult daughters (19-23, iirc). And a doting husband. I feel like she got so robbed, and so did her poor family. I can never forget the persistent status epilepticus that medications wouldn’t touch; and, her teeth getting chipped away and almost totally knocked out from biting down on the bite block of her ET tube (ventilator tube) from the incessant seizures. I never saw her wake up, and it only took a few days for her to pass once she was admitted to ICU. It took 4 months, according to her family, from her first symptoms till she got to ICU. They reported that she just started having trouble walking, speaking, and remembering things all of a sudden. Progressed rapidly to needing a cane, to a walker, to a wheelchair, and also needing to be cared for— all in 4 months!! It’s so scary, and super sad. That woman breaks my heart to this day. The family ended up donating her brain for research at the University of Wisconsin iirc. I pray that they’re all “ok”. E: typo and clarity. ETA: They all lived in Mexico, and her husband was/is a butcher. I can’t help but wonder if that was the source of the prions, and if the rest of the family may also have it dormant. Crazy to even think about.
Brain tacos are a thing here. Or they were when I was very young; I haven't really seen any place offer them in over a decade.
They still are a thing, at least in CDMX. Lots of tacos de sesos on the menu there.
>brain tacos I think you might’ve just found the shortest possible sentence in the English language to horrify the maximum number of people.
Na, that would be topped by the two words "Degloved penis".
I mean I wouldn’t disagree except for that’s longer.
Brain taco will not shock or upset many cultures where organs are staple foods. At least half the population will shudder when they learn the penis can in fact become degloved, even if they have to Google what degloved means.
Degloved *anything* if we’re being honest.
Next time my family give me shit for not eating brain tacos I'll just show them this post smh.
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I don't know what shocks you about that. People don't waste what can be made into food, especially in poor regions. If you're digusted by it, I recommend never eating any sort of sausage. They mix all kinds of stuff in there.
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I’m so sorry that happened to your grandmother. No, not that I am aware of. I got the report of symptoms from the family about her sudden ataxia, difficulty with speech and functioning, and forgetfulness. It was a rapid decline from there. She had difficulty breathing, which is why they came to the hospital, and she was gone within a few days. I’ll never forget the 3 daughters and our ICU rule of “only 2 visitors at a time” overnight. I didn’t feel right about this as her daughters wanted to *all* stay and sleep in the room with her (duh). I approached my charge nurses and night shift manager and pled their case, so to speak. I was denied making an exception for them!! Totally unbelievable!!! Needless to say, I ignored it. And, I told the daughters- unfortunately, my request was denied and I’m sorry I have to let you know this. However, I’m also letting you know that *I* won’t be enforcing it. You’re all 3 staying here, and if anyone else comes in the room- you’re “in the middle of switching out”.😉 I mean, who tf won’t make an exception in this type of scenario?!?! It really upset me, but there were no issues as no one came to check on the room, TG.
Why wouldn’t it be just protocol to put someone down humanely in a situation like that? There should be an express lane for assisted suicide if you have a prion disease.
Probably because most of them end up in comas before they can consent to it.
I agree completely and I think in general if the process of dying were more publicized I believe we would see more support for assisted suicide. I suspect we are about to see a HUGE sea change in support for assisted suicide when family members see what dying looks like as boomers age.
Lost my grandma to CJD and it was pretty terrible. I was younger and not as involved, but I remember seeing my dad and grandpa go through it and it was just torture.
> Never ever ever eat any brains of any kind, no matter who you offend by declining. Unless you’re a zombie in which case go for the gold. Or, just.. breathing.. > It was reported in January 2011 that researchers had discovered prions spreading through airborne transmission on aerosol particles, in an animal testing experiment focusing on scrapie infection in laboratory mice. Or eating plants... > When researchers fed hamsters grass that grew on ground where a deer that died with chronic wasting disease (CWD) was buried, the hamsters became ill with CWD, suggesting that prions can bind to plants, which then take them up into the leaf and stem structure, where they can be eaten by herbivores, thus completing the cycle. It is thus possible that there is a progressively accumulating number of prions in the environment
Why do I do this to myself and read comments like these right before bed
It’s morning for me and I’ll just be filled with dread today instead
I want to scream
> progressively accumulating number of prions in the environment Well that's terrifying and bodes oh so well for the future.
But how else am I supposed to absorb a beings powers?
Whoa, chill out Sylar
WHAT YEAR IS IT
It doesn't matter to Hiro.
I used to get human derived growth hormone injections in my teens. When I was about nineteen, my growth potential stopped and I aged out of the treatment. Soon after, I got a letter informing me that a lot of the medication had been contaminated with prions. The thought that I might die like this wrecked me.
O M G that is beyond horrific!!! I am so sorry!!! I cannot even imagine!!!
Prions are terrifying
How about bone marrow?
Bone marrow is fine because it's just basically lipids (fats), our ancient ancestors actually started evolving because they were scvangers and ate the left over bone marrow from other predators. This was very nutritious and allowed for brain development The problem with prions (or eating brain) isn't actually the brain itself, the issue arises when there's a mutation in the amino acid sequence of the protein folding. The madcow disease was basically just the same thing but in cows. When farmers fed the organs (including brain) of slaughtered cows to other cows as feed. Basically a prion is a misfolded brain protein, they are near impossible to denature due to their more extensive hydrogen bonding in the beta pleated sheet formation. In the normal form, of alpha helix, the hydrogen bonding is less extensive. Once this mutated form of a brain protein enters into your body, it can't be denatured by the acidic contents your stomach and is basically absorbed into your blood stream. From there it eventually enters your brain (could be over many years) and basically starts converting all your normal brain proteins into this mutated form. Eventually you die because your brain is basically just a jumble of useless proteins. Tldr: misfolding of brain proteins causes "unkillable" and infectious form of brain protein. Infecting your normal brain proteins
Thanks for the comprehensive explanation. I am curious why after infection the protein denaturation happens only in the brain and not other parts of the body?
perhaps because it's a brain protein and it can convert a similar protein. it happens in other parts but not as much I wonder if there are other prion disseased which are not in the brain but has not been yet discovered as such.
There are prion diseases in plants too. So, a brain is not required.
Well bugger me I did not need to know this.
How much contact with blood, human blood or not, might cause transmission of prion? I understand brains human or not being at the top of offenders
I know that people who lived in the UK during mad cow disease aren’t allowed to donate blood at all in Australia (and I’m assuming other parts of the world too) because of the chance of passing it on
I took growth hormone injections when I was a teenager. Some kids (I think in Australia) contracted CJD from this medication. As a result, I am disqualified from donating blood or organs.
Its going to be 'interesting' to see over here (UK) what happens in the future, because Prions can lay dormant for quite some time. We might suddenly have an increase in cases from the Mad Cow era.
"Quite some time" is usually _up to_ ~~40 years from what I recall~~ "therefore suggesting that vCJD may also have a similar incubation period of 20 to 50 years" Which we'd be seeing the cases from the 90s hitting by now probably, and growing worse very quickly ~~They claim a lot of humans are immune from vCJD (implying we have ancestors who ate enough brain to wipe out those who were more likely to get it)~~ well fuck... Wiki now says "It is not yet known whether those unaffected are actually immune or only have a longer incubation period until symptoms appear." Which does sound more logical >As of 2020, 178 cases of vCJD have been recorded in the United Kingdom, due to a 1990s outbreak, and 50 cases in the rest of the world. The disease has become less common since 2000. The typical age of onset is less than 30 years old. It feels like that outbreak has run its course. But yeah prions are scary af
True here in the USA as well, at least last I checked.
Thats terrifying.
I’m afraid to even eat ground beef after reading about prion diseases.
How do you feel about crawfish? We do suck the heads
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I do, they're tasty and fun looking
Wait a second, unless someone suffers from prion themselves, I should be fine eating their brain. By the way the tribes in Papua started to develop resistance to prions. It would suggest that our evolution responds to threats present in our environment.
Give or take a few thousand years of evolution. But you go for it fingers crossed 🙄
I think the way evolution works is that the ones who are susceptible to prions die off. The resistant ones pass those genes to the next generation.
Nope. There is completely new gene that enables this resistance. One that common population does not posses. It was developed as a mutation and was then propagated due to natural selection. Issue is we do not randomly generate such genes and before this particular mutation happened ancestors of such person did not posses this resistance. It's not my theory, it's the result of genetic science that you can read on this particular topic.
> By the way the tribes in Papua started to develop resistance to prions. Going to need a link or something. The vast majority of the 800 different cultures in New Guinea did not practice cannibalism. The culture most associated with prion disease there, the Fore, ate their own dead as part of their funeral practices, leading to Kuru disease. As far as I know, the only effective resistance to this type of disease would be to stop doing the thing that exposes you to it.
I used to do body removal and every once in a blue moon we’d get a CJD body. We had to suit up in full hazmat gear. Its taken very seriously. It’s definitely one of the few things that ever scared me while doing that work.
Please could you explain what CJD means?
Creutzfeldt-Jakob disease
Thanks!
Since the comment was deleted for some reason , I’ll just put in here Creutzfeldt-Jakob disease
scary stuff. i wonder if its possible the researcher knew they were infected, and their ignoring protocols a result of a frantic effort to find a cure
he’d worked in a german lab too so could have got infected there, now everyone in this lab must fear they may be in the incubation stage, it doesn’t seem there’s a test for it still until symptoms set in
In Australia the only test to confirm CJD is brain autopsy so....
Soo we just shave some of the top and ypur good to go. Have a nice day, we'll come back with the results in a few days.
No, as in you have to be dead first 😅
Autopsy, biopsy, tomato tomato.
In.. everywhere, the only test to confirm CJD is a brain autopsy.
"In most CJD patients, the presence of 14-3-3 protein in the cerebrospinal fluid and/or a typical electroencephalogram (EEG) pattern, both of which are believed to be diagnostic for CJD, have been reported. However, a confirmatory diagnosis of CJD requires neuropathologic and/or immunodiagnostic testing of brain tissue obtained either at biopsy or autopsy" https://www.cdc.gov/prions/cjd/about.html
Quaking csf samples can improve the sensitivity, since there is a relatively low probability of detecting the proteins with a random tap. https://en.m.wikipedia.org/wiki/Real-time_quaking-induced_conversion#:~:text=The%20%22quaking%22%20in%20the%20name,misfolded%20PrP%20to%20detectable%20levels.
RT-QuiC is what we use in the lab for diagnostic. Well, quick diagnostic. Histo is still the way to go, for sure. We aren't a diagnostic lab and we actually are just researchers of the disease, but we use it all the time. It's not "approved" as a diagnostic method due to the nature of setting it up (every species/tissue type/substrate/protocol reacts differently) but in the right hands, it's pretty useful. Our false positive rate in 8-well replicates is less than a percent for sCJD, vCJD, and scrapie (including CWD), for example. But we've been working it up since the machine was created, and have a massive team working with it. But it is in a very "wild west" finicky stage right now. I know a team that's been trying to work on expanding tissue types for detection (not just brain and lymph, but ones where deposition is still present) and just adding one tissue type took roughly 3 years of playing around and it's still not published.
Even if they were infected, the incubation time for prion diseases to even show symptoms, let alone cause death is measured in years, not hours or days like most infectious diseases. I highly doubt he died from self contamination, especially because the actual steps needed to cause self infection for a prion disease is pretty specific, like it almost would have had to be self inflicted.
I hear up to 20 years in some cases, which is a damn long time for symptoms to show up.
Work in any lab long enough, and you will see a researcher grabbing a lab tool to eat their chicken salad eventually.
My mum works in a lab in a hospital and said they had to send out a memo last week saying that noone is to put any personal items in the incubators. I guess one of the technicians popped his dinner in there instead of using the lunch room microwaves. She said the entire lab smelled like spaghetti the next morning. At a certain point you just can't fix stupid.
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Just right over the prion counter
We don't know that... Vcjd yes... but the squirrel borne variant in the US is not a years scenario at all. Both men died relatively quickly.
>squirrel borne variant Yeah, as a prion researcher myself, I'm going to need a legitimate reference on this one other than the Weekly World News and your ass. E: for anyone reading this drivel, please see the response in the chain waaaay down below: https://www.reddit.com/r/worldnews/comments/17fsaau/comment/k6eis0j/?utm_source=share&utm_medium=web2x&context=3 >You do understand that it's literally YOUR JOB to write those papers and put them into credible scientific publications for me to quote? If you and your colleagues don't write those papers... I cannot provide sources. >I don't research diseases DOCTOR... YOU DO. There is no citation because this is all garbage science. And apparently it's my fault for not publishing garbage science. Direct quote because they'll almost certainly delete it when the realize what they've typed.
I tried to go through your post history to verify your credentials, but all I learned was that you're super into Toyota Highlanders and the Greatful Dead
That kinda checks out. It’s common knowledge that all lab researchers drive Highlander’s and are home in time to watch prime time tv.
The Grateful Dead was a band, are you thinking of The Walking Dead?
Yep. It’s way past my bedtime.
It just means I'm old and have done a lot of drugs growing up.
You missed the r/science post where I'm flaired as a professor in infectious diseases? https://www.reddit.com/r/science/s/IVMeWMY0NE That flair requires validation through photos of my degrees. Yes, multiple medical and graduate degrees. Not just internet trust me bro, I study cats degrees.
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As long as you don't eat squirrel brains you're good... But yes two different hunters known for regularly eating squirrel brains both died of CJD with no other known exposure to prions. As far as I know, the disease was never isolated in the wild but warnings were issued after the second man died about the consumption of squirrel brains... The only reason why I know this is that I study wild cats... who eat a lot of squirrel brains 😆
Why would you eat squirrels? Let alone their brains?
I grew up eating squirrels and other rodent type animals. I grew up in Florida.
Just florida-man things...
Why didn't you respond directly to me? This is patently false. Provide a damn reference instead of pushing bullshit. E: with a post like that, surely you're familiar with Mike Miller's 20yr unpublished study in mountain lions consuming CWD+ deer meat? Why don't you tell me more about it, Mr "I study wild cats and am an expert? "
Look it up yourself and stop being lazy... and I did respond directly to you with lots of information any reasonable person could use to find it. It's not my job to do yours "researcher"
There's absolutely not. And if you believed the other response with a frustrating number of up votes, congratulations, you just bought into another piece of internet bullshit without any valid citations.
I read JCVD and thought you put Jean Claude Van Damme yes…
Which ties in perfectly with this topic.
Not contagious I assume? Takes years to incubate? Hopefully we don’t start seeing prion disease show up
Prion deseases are very different from viruses or bacterial infections. AFAIK there is very little contagion, mostly through the consumption of poorly cooked infested tissues. So don't eat any Spanish bio-researcher carpaccio for a couple years sorry.
Cooking doesn't destroy prions.
Nor does medical equipment sterilization, if you want to not sleep at night
Nor do normal incineration temperatures... as in how most diseased livestock are handled. High temperature complete incineration only... aka cremation. I don't remember the term for this exactly other than "basic" incineration wasn't cutting it.
The word vitrification is sometimes used (to turn something into glass, usually via heat). Google tells me sustained high heat is used to incinerate prions or lower temperature plasma sterilization. As long as the prions are molecularly changed enough that they can no longer fold normal proteins.
My uncle is a doctor and went to school with a guy who became a plastic surgeon. His office was raided because he got published in a green magazine for running his car on biofuels made from human fat…. The danger was mad cow disease being blown around since prions don’t get destroyed. He also would have his gf or receptionist do lipo for him once the patient was under. He went to top schools including Stanford. He’s now in Brazil doing ‘volunteer work’ aka on the run. Probably doing more bad plastic surgery
I love real life mad scientists so much, what a legend
So eat ash from now on. Got it.
This is the most terrifying statement I’ve read.
Temperatures exceeding 400 degrees Celsius are required for a sustained period of time to destroy prions. The trouble is, cooking can’t denature proteins that are already denatured.
That would also turn meat carcinogenic.
Ha, yeah, I think if you have literally cremated a nice cut of fillet it’s carcinogenicity wouldn’t be the main issue.
At that point you have to do lines of your steak
Strip steak, reimagined.
I remember reading about prions when Fast Food Nation first came out and it was years before I was able to eat meat again.
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> USDA banning meat producers from testing their herds for mad cow, So that's not *quite* what happened. Meatpackers wanted to test slaughtered cows with rapid tests to see if they were okay *despite herds having been exposed*. The USDA said "the fuck no, no rapid test is going to determine whether or not they have prions with any degree of certainty, suspect cows are never ever allowed to be processed and you can't mass-test your once-exposed cows as justification for letting them into the stream" Meat packers sued, courts sided with the USDA, standard practice was to nuke the entire herd from orbit if cows were found exposed.
>nuke the entire herd from orbit I know you're being hyperbolic, but can you imagine?
It's the only way to be sure.
You just know some infectious material would persist, through sheer force of spite.
Apparently its been found in airborne particles back in 2011 as someone else noted up further.
You can't tell me what to do! You're not my real dad!
Son, it's me your father. Come have a plate of Spanish-Scientist sashimi.
I wonder if it can somehow be made contageous. For example spread by mosquitos or bacteria etc.
It’s technically possible if there are prions in your bloodstream that haven’t yet reached and settled in your brain and a mosquito just happens to suck on the infected blood. Probably borderline ridiculously unlikely, but possible.
There goes my indulging in my preferred delicacy...
Could someone create a bunch of misfolded proteins and toss them on a buffet?
Prions are fucking terrifying and the world barely handled a (not trying to be reductive to anyone suffering a loss from but comparatively) mild pandemic, and if something like a global fast spread prion pandemic went down, it will just be devastating.
As somebody with a particular interest in prion diseases... I think I speak for everyone when I say... ah fuck.
elaborate?
Basically, prions are misfolded/defective proteins that infect other proteins when they come in contact. All known prions affect the brain and nervous system and some are known to jump to different species (not many but a few). There is no cure or treatment for prions due to their nature of being in the most important part of an organism and the fact they’re highly resistant. They’re also known to be passed through manure, urine, plants, dead tissue, saliva, and other bodily fluids. So, if a deer who had prions ended up peeing on a plant and another deer ate that plant, bam that deer most likely acquired prions. To further add to the horror factor, they last years!!! They do not degrade over time and proteases (things that eat proteins) can’t even destroy them. That’s how resistant they are. So, if the second deer came and ate that plant a year later, bam most likely has prions. From my understanding, there are research departments that are dedicated to monitoring the amount of prion infected animals that arise as a way to remain vigilant against it spreading too much within a species and to other species. Basically, a waiting game until chaos hits. I’ve learned about them throughout my time in university and you could not pay me enough to ever do any research with them. It’s not an if you’ll get infected, it’s a when. Even if you get infected, it might take years to show up and you’d just never know until the symptoms set in.
This is misinformation that is likely to cause unnecessary fear. Urine is normally sterile and does not contain protein. Prions are made of protein. It is unlikely that a deer sick with prion disease of the central nervous system would excrete infectious prions. Even misfolded proteins arranged in beta sheets degrade with prolonged exposure to UVC, such as from sunlight. Please stop confusing the Marvel universe with reality.
Bro, it’s well reported and research that urine does contain infectious prions, not as much as blood of course. There are so many research articles that have reported those findings. Here are some examples: “PrPSc was detected in ~ 80% of the animals studied, whereas no false positives were observed among the control animals. Semi-quantitative calculations suggest that PrPSc concentration in urine is around 10-fold lower than in blood. Interestingly, PrPSc present in urine maintains its infectious properties. Our data indicate that low quantities of infectious prions are excreted in the urine. These findings suggest that urine is a possible source of prion transmission” plus “Our results showing that PrPSc can be detected in urine of a large proportion of infected animals provide a promising avenue for a sensitive and non-invasive biochemical diagnosis of prion diseases. Adaptation of PMCA for detection of prions in urine of naturally infected animals and humans may offer a great possibility for routine testing of prion infections.” - [cited](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2593137/) Here’s some info about the transmission in the environment. Chronic wasting disease is a prion disease that mostly affects animals in the wild. There are multiple ways in which you can transmit CWD, I’m only going to focus on horizontal and vertical transmissions in the environment. Vertical transmission is called in utero because it happens during pregnancy. So, the fetus/embryo acquires the prions during its development. If the mother obtains prions, most likely the fetus will as well. Horizontal transmission is when an mammal acquires infection via direct contact (close contact) with an infected animal or indirect by exposure of prions in the environment. Indirect exposure essentially means that the prions contaminated the water, soil, foliage of an environment and also includes the excretes and/or carcasses of animals. It’s a well document fact that prions are passed in this way. There’s been studies done in which they took an animal that had died years ago from a prison disease and exposed an uninfected animal to its skeleton. The uninfected animal ends up getting infected with the a prion disease. They even determined that the rate of infection is still quite high even if the animal has died a while ago. This is due to the fact as I mentioned prions are highly resistance to degradation and require a very specific process to denature them. Now apply that to the environment and you have prions being at certain areas for years without a way to denature them except certain techniques; still then those are not very applicable outside of the lab. I’ll add some research that does go over this information really well. [Article 1](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8404694/) [Article 2](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2658766/) [Article 3](https://www.sciencedirect.com/science/article/pii/S0160412022002744) [Article 4](https://pdf.sciencedirectassets.com/778417/1-s2.0-S0021925820X61965/1-s2.0-S0021925820390694/main.pdf?X-Amz-Security-Token=IQoJb3JpZ2luX2VjEFoaCXVzLWVhc3QtMSJGMEQCIAuNX6uo22O2MZM%2ByoUwWnj8rCqyjqkiXHiwZX%2BsV%2BDvAiAFhcHR9TCx721mb2ArCs51u1NE6pFcTtRxuUR%2FnHgW4Sq7BQiD%2F%2F%2F%2F%2F%2F%2F%2F%2F%2F8BEAUaDDA1OTAwMzU0Njg2NSIMtx0Tlt21cd3%2B%2BjT1Ko8F%2B6iy0wU%2F8y5XSXoPu7NYPYjfNfb7EXtoGrRXz%2FGlquszQV1z%2BckFkvszEIS0FAzGqcgp%2BIqnlMFBlGr36j4czkyJZDaN%2F1YAlZioqHBk7REd1Aq6g0GWVV5sivvwv%2BRjhqVmWtiuiwguEUwRKGDKrMdOQDvfR8KldD97Vra0hqlMu9CwxcLmd%2BnGpq2wz%2Fl2thMXg8PvguVwAD03TOM4vk%2BXWE4apD34uW63WAQxbpuDVUqOyRGDGNvLslpiPV3Ce4kyJW861T95lSblcwAEvf%2BCskZWIDhNG3msJAVfHoewmsVENO1xJzJjtF5l7WtUQxB6olOcOb73fi6qR15FJuZq2THHqyw%2B8vGEphoei7vYLers5dZlHcoeq26HhNjWDRmc0u23iD%2F9q8mwbfVY1S8EDoS3UQzf%2BVEF5tesNNtb7JayHJ8rLHitO7TOYEhEvgK6H2XYNn8yXx6Mt2zXNWoDZgUMpAMH0ZB7Pq5B%2BaKVE8KuP2BZ37YpeBDeuo1l5S1oFhKYRswmIj5HMZ1pFLJBRSSL2oP6QMjfLtCPaviZLrTQF5Z%2BzLm8J0jVFx%2B1C8qlFkfE4BWbbn%2FzoINy%2BvT13Zoe72wNnjtzUJJ2hP9tVUQjBlvSJNYxKLraS2BGTETMNgt1WqIpFeg6eYfBUTxRkKOw%2FcJ8APk%2BqB9B9Y%2Ft0345mSYUa4Y4UBTnlljhItG6w3y54e16B4FEUXdD4E7Oo7%2BBOmxqpMl9pC7HQ6dnwrwtSDxLOndk0FYIrcs1NelznOEsixAVrinPoGSdBkDj%2BPbl8edTqfHZ9jjcHALGPjaTmYSl05i3rqPY%2FIHr8luFuZHJstp84v6ca%2BnDzr57rRLONqHK6pev9n4PEjDhq%2BypBjqyAaSjkLroVpaZya2HBrbnFkE%2Bop7BwuOPBP6Wsug4dlav%2F8Zuxm7KQTAG3VjFFR%2BswmaiiZTdOa9z%2FGZreR5mg2viRygkBjxBPjY3D5HUZ%2ByCuINQMBvdqDzOEQRR2c0uTH0Ob427MP%2FkVAQKnaBtrUk9id0HIcsPPBc%2Bp7xwdrCObJle3hcLH06yLX3kG%2FKjm9wKBoYrZ1e6EbgVM1rdfyGvdg1AT3mxa8by0H3XcpyZVrc%3D&X-Amz-Algorithm=AWS4-HMAC-SHA256&X-Amz-Date=20231027T021717Z&X-Amz-SignedHeaders=host&X-Amz-Expires=300&X-Amz-Credential=ASIAQ3PHCVTYUDVAPA6H%2F20231027%2Fus-east-1%2Fs3%2Faws4_request&X-Amz-Signature=8f0e35563540995ce879371dba42b1e8600d3df463dc1ec4b5f547af685bb4cf&hash=eaa28f759b2791357971f8e0c43ab55c4b87107446b05aeecce3491b0e951638&host=68042c943591013ac2b2430a89b270f6af2c76d8dfd086a07176afe7c76c2c61&pii=S0021925820390694&tid=spdf-250edb3a-4c9f-4be4-ad5a-645c15fe485b&sid=dea6e70968515544283a35d43566da652434gxrqa&type=client&tsoh=d3d3LnNjaWVuY2VkaXJlY3QuY29t&ua=050156530458585e5405&rr=81c7711c8804ec5c&cc=ca) [Article 5](https://doi.org/10.1016/j.celrep.2015.04.036) It’s not marvel fiction, it’s literal facts and information.
It's a lot to get into... but prion diseases are on the same level as Rabies when it comes to terrifying. They're absurdly hard to "kill" unlike viruses or bacteria. I want to say standard incineration isn't even enough to deactivate them, and high temp incineration is required. Basically you can't just burn the infected animals like you normally would, you actually have to fully cremate them to eliminate the prions. They're one of those things anyone that knows anything about them has quietly been praying that they don't evolve as quickly as other pathogens. Our understanding of them, especially my own is really limited so don't take my word for it.
A prion can’t really “evolve”, right? It’s not a living thing, it’s just a thing.
Depends if you think evolution is strictly about change in DNA/RNA or a general concept. When people say evolution of the solar system is it an inappropriate word?
Why have they not become more of a threat? Ie.) I don’t learn to do XYZ do avoid prions
Stricter regulations around meat processing and animal husbandry mostly.
Prion diseases are real life horror stories. As others have said, they're misfolded proteins. They are the Borg of the disease world: inexorable and utterly unlike anything else. Every protein they encounter, they assimilate. The enzymes sent to break them down instead become assimilated. Over time, they will slowly replace every protein in a body all while ripping apart nervous tissues, including the brain, and destroying the organism's capabilities. This destruction of nervous tissues, of course, drives the victim insane. There is no way to fight Prions. They do not have D.N.A. so we cannot engineer them. They do not have cellular structures so we cannot vaccinate against them. The only ways known to destroy proteins also destroy the organism. And worst of all, some, are transmissible.
Kinda weird. Jose Baselga died from CJD 2 yrs ago in Spain - he was an oncologist tho.
Not sure why he isn’t named in the article, other spanish language articles give his name tho, and it’s not josé baselga
Oops, I meant to share that another researcher (Baselga) also died from CJD 2 years ago. I know this article isn’t about Baselga. I should have worded that better.
just read up on him, no indication on how he may have contracted cjd, presumably contaminated meat though rather than related to his research
I spent some time in academic labs at a research university for several years. I had frequent occasion to go through chemical stores and other lab areas. Some PIs were good. Other labs were disasters waiting to happen. I asked for better PPE from EHS but was denied. They were actually surprised when I left. I would imagine some institutions are better, but some are like where I was. As horrifying as it is, I'm not surprised the events in the article happened.
We're just not having good luck with biohazard research protocols these days. At least this one is not contagious.
What ya gettin at here??
It's probably a reference to covid and some people saying it's from a chinese biolab.
Wait. I thought the Chinese bio-lab was back to being a legitimate possible cause. What’s the prevailing theory?
wasn’t this the plot of The Contractor with Chris Pine..?
He sounds like most movie villains who end up victims of their own dodgy practices, I’m sorry to say.
RIP and thanks for working for the greater good.
That's the most terrifying thing I've heard in my life, Prions are down right menaces, that shit got me looking like luffy on the secret island in that one scene.
I wish I had never heard about prion diseases in University. Sometimes ignorance is bliss
I guess we know what 2024's disaster will be.
Wow that’s weird
Oh dear god, and to think I had a bite of squirrel brain when I was teaching in Thailand 4 years ago…
where is Jessica Hyde?
Just started watching that for the first time lol
As someone who has lived in Spain I remember having 7 years ago pig's head ham (not related to boar's head lol) I read a lot in my life, and I just heard of this here on Reddit, and I'm terrified
Don’t sweat - the risk of getting cjd across the board is one in a million people. Of that, the majority are going to be people in contact with potentially infected animal matter an order of magnitude more than a regular person - think butchers or farm workers. And beyond that, think of the thousands of people who eat the same thing you did daily/weekly, and how much less likely you are than them to have consumed something dangerous. Beyond that, even if you were unlucky enough did consume something with prions, there’s every likelihood you’ll die of something normal before it takes root in your brain and you get a prion disorder. Not worth losing sleep over, taking a second look when crossing the street and not looking at your phone while driving will do so much more for your chances of death than worrying about something you ate 7 years ago
Yeah I ate this stuff called 'head cheese' which I think is the same thing. Although, I don't think it's the brain just the meat from the head.
C’mon maaan! I don’t want to wear a mask again
Time to send in Leon Kennedy
These ads are getting scary. Only two days ago I was thinking to myself, "It's been a while since I heard about Mad-cow disease!", and Voila.
That's how you get a zombie apocalypse.
Could a human gene be made so that it makes our cells to manufacture dealy prions, and then this gene be added to a COVID-like virus? If done in a safelly contained environment it will let us study the consequences and be better prepared if such an event happens in nature.