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[flagged] Prions (countere.com)
155 points by pccampbell on April 19, 2021 | hide | past | favorite | 168 comments



Counterpoint: The Fore people of NZ had kuru from ritual cannibalism. Then they stopped. Eventually, the disease stopped too. Prions are nasty, but we've had 3.5 billion years of protein folding and none of it has ended the world yet.


This comment is spot on (I'm a former prion researcher).


What do you make of CWD?

That one scares me more than current known human prion disease. Mainly because it seems to spread really easily. Other prion diseases seem to practically require cannibalism to spread.


Cwd is essentially the same as bse and cjd and scrapie. It's actually the one that you have to worry about the least. For unknown reasons, there is not species crossover between deer and cow/sheep/humans (I was briefly investigating the molecular mechanisms for this but finished my phd before getting results).

As for why the grazing animals can spread prion, my guess is that the prion protein is highly concentrated in the tongue, and grazing animals are likely to be able to pass the protein down to the ground via the saliva. Scrapie and bse are cross-transmissible

Incidentally, you probably shouldn't eat beef tongue.


The CDC disagrees with your assessment. Do you have any sources about this?

According to the CDC, CWD is spread NOT just through saliva, but also feces, blood, urine, etc.

In addition, it CAN spread to Squirrel monkeys. The long-term affects on macaques is still being established (on of the reasons kuru took so long to discover was that it took decades for the monkey to develop symptoms).

If exposure is dangerous to humans, what could we do? In the areas it has been in, it is spread everywhere the deer travel as they shed bodily fluids into the soil.

source -- https://www.cdc.gov/prions/cwd/transmission.html


So I should have disclaimed that my information is as of 2009, which is the last time I touched this stuff, but that site said there was not successful transmission into monkeys in another experiment by the lab. I mean these experiments are extreme, high dose boluses of material straight into the brain. If at least one experiment failed to do that into monkeys that says something, even if they managed to get it in a later one, unless there was some revelation that they had done it wrong the first time.


"there is not species crossover between deer and cow/sheep/humans"

I thought the theory was that CWD started in a domestic herd that was raised in an area that previously had scrapie infected sheep?


These things can also happen autogenously (there is sporadic cjd) so who knows.


Wait, wait, wait. Your advice seems contradictory: "there is not species crossover between deer and cow/sheep/humans"

and

"Incidentally, you probably shouldn't eat beef tongue."

Can you clarify?


Sorry, the grouping was unclear.

There is not deer <-> sheep, deer <-> cow, deer <-> humans.

There is cow <-> sheep, maybe cow <-> human. I don't know off the top of my head about sheep <-> human

If there had been, the us food supply would be trashed. Cwd is highly endemic among wild deer and wild deer co-graze with cows all the time.

Also unknown if the relationship can be transitive. There might be a species X where deer <-> X <-> human.


Scrapie appears to have been the ultimate cause of a human CJD outbreak:

"...in 1996, with the recognition in young people in Britain of a “new variant” of Creutzfeldt-Jakob disease (nvCJD, or the Will-Ironside syndrome) that has since been traced with near certainty to the consumption of tissue from cattle infected with spongiform encephalopathy (BSE), they having in turn consumed meat and bone meal contaminated with rendered sheep carcasses infected with scrapie... There are currently just over 30 verified cases of nvCJD, and four to five new cases a year: whether they represent a small group of susceptible people or are the leading edge of a major epidemic is still moot."

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1114482/


No lengua tacos? What will I get now?


Cabeza. Which is usually the cheek meat. Avoid sesos, of course.


But lenguas?!?


CWD hasn't jumped to humans ever, correct?


They don't really know, but the evidence suggests that it doesn't, or at least hasn't yet. There have been a few studies following up on groups of people who consumed CWD positive deer.

https://www.usatoday.com/story/news/health/2019/02/21/zombie...


No. But Mad Cow Disease (also caused by prions) sure did. More info: https://en.wikipedia.org/wiki/Bovine_spongiform_encephalopat...


How old are these proteins? If the do spread and remain dormant in soil they would have been a threat for entire existence of life on this planet. Exponential growth would only cause every place, especially a bio-reach locations to be full of it as food chain making more and more of the protein and animals remains are not handled in any way in the wild.


They're old. Yeast have genes that transmit information generationally via the same mechanism. Probably the same "exponential" mechanism is involved in diabetes, alzheimer's, melanin storage (not a disease, the actual normal process).

Proteins, eventually degrade, even prions. We probably have an enzyme that accelerates degradation of them (my bet is it's insulin-degrading enzyme).


I don't think proteins will live forever in the wild. Between bacteria, fungi, and just oxidation, I'm pretty sure a protein just sitting out there on its own is going to have a finite half-life.


It appears that the first mention of scrapie in scientific literature is 1755, and the disease increased with inbreeding (and lessened when this practice was stopped):

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1114482/

The wiki also indicates that a sheep vaccine distributed in 1935 caused an epidemic, as it contained contaminated neural tissue:

https://en.wikipedia.org/wiki/Scrapie

It also seems to me that the recent mRNA vaccines could be repurposed to target a section of a prion, so the immune system could clean them from the blood. An interesting question would test mRNA vaccine effectiveness in the cerebrospinal fluid, where microglia would be required to perform this function, as macrophages (and friends) are not present there.


This is a very interesting idea! But, is it known that macrophages are able to break down prions? My understanding of the problem is that part of it is that they're just quite hardy.


Even if they can't, it's probably better for them to build up in macrophages, dendritic cells, etc. rather than in neurons.


Disagree. Every single antibody alzheimer's drug that has targeted the amyloid plaques and none of them have succeeded.

You're just as likely to elicit an immune response to the prion plaques, stabilize them, and recruit inflammation to the point of deposit, making matters worse.


Curious, I had heard of research where the brain of a sheep that was infected with a prion disease (not sure which), was burned to ash, and that ash placed on the exposed brain of a healthy sheep (via trepanation?), and it also developed the prion-caused disease. Is this anecdotal nonsense, or is it close to a real study done on prion diseases? Thanks!


That's real. Moderately high temperatures do not effectively destroy prions/amyloid.

Note that "burning to ash" is not that hot.


Wow, thanks for that! I don't know why I thought it would be so hot when I used to weld and cast metals. I remember when I was a kid we used to say something like, "I'll burn you to ashes" when playing. Still amazing that you can burn it and it's still potent.


What are your thoughts on the risks of AlphaFold and prions?

It seems like prions don't have much chance to evolve/mutate naturally since any change makes them unable to duplicate. However with these simulation systems, are man-made prions a concern?


> naturally since any change makes them unable to duplicate.

That's not true.

Don't worry about alphafold. Even if it's a really good extrapolation engine (and not a really good interpolation engine) the amyloid fold is unusual enough that I doubt that alphafold can heuristically solve structures much less assess energetic or kinetic factors (which is not what it was designed to do). Note that I am an alphafold optimist and am on record here saying that I consider alphafold to have solved the basic protein folding problem. More to the point, we don't have many reliable solved structures of amyloid folds, it's clear that they are heterogeneous anyways, and iirc no one has bootstrapped an infectious prion in vitro, which means we don't know exactly what makes something infectious... We can make prion amyloid in vitro but it isn't infectious unless it was seeded by an infectious prion amyloid.


As a former prion researcher, do you eat meat or do you have reservations around that?


I don't have reservations about it. I'm also not generally afraid to die.


Was curious if your research had tainted concerns around eating meat similar to commercial fisherman not eating fish due to work environment revelations.

Glad to hear you aren't generally afraid to die though - sounds like a good stance to have.


Globalization is a fairly new thing (on the 3.5B year timescale at least), allowing for the spread of any disease quickly. Whereas isolation and evolution has saved us in the past, it might not have the necessary time in the future (imagine if Covid had been even more deadly, for example).


Prions don't really spread on their own though, yes they are extremely hardy and can survive in soils but that doesn't do anything, if it did and isolation was the only solution we'd have thousands of prion-infested red zones where humans couldn't set foot.

> imagine if Covid had been even more deadly, for example

It'd have been taken more seriously, and it would have had a higher chance of burning itself out. Covid's such a pain in the ass because it hits such a sweet spot, of spreading fast, being highly infectious, and being benign enough (with many of the infected spreading it asymptomatically). For the hell that it is, the one "saving grace" of Ebola is that it debilitates and kills fast enough it's very hard for it to spread, especially as it doesn't have great transmission vectors.


What I remember from middle school biology is that nutrients flow in a cycle. Organic matter in the soil will be composted and consumed by earth worms, funghi and microorganisms.

And to them, prions are just organic matter like any other.


In addition to the points other people made, proteins are really a whole different thing from viruses in terms of numbers. You make so many proteins every moment, at this point every possible screwup has been cooked up somewhere.


If COVID were more deadly like Ebola it tends to not have long gestation periods where it can be spread without symptoms.


tends to is not going to cut it in this instance. If 99% of diseases are either easy to spread, or deadly, we still need to worry about the 1% that are both. It only takes one such disease to cause a wipeout like event.


I was going to add a flow up edit to add more clarity to my answer; the point is if 50% of people are dying from an airborne disease, getting to zero is going to be treated with much more importance than COVID with a ~2% (? maybe) fatality rate. People who think masks are a political tool to control us suddenly are less paranoid about that (and vaccines, and social distancing) when the bodies are bing piled up in the streets.

I think we'd probably handle a more severe disease with much less flippancy than we did COVID and we'd have to treat lockdown and other tools with much more reverence in such a situation.

This does happen with Ebola outbreaks being managed in Africa now so if they can do it there I think we can do it here.


0.7%, not 2% (according to the US CDC). Which does make it "worse" for the reasons you point out.


> getting to zero is going to be treated with much more importance than COVID with a ~2% (? maybe) fatality rate. People who think masks are a political tool to control us suddenly are less paranoid about that (and vaccines, and social distancing) when the bodies are bing piled up in the streets.

I agree with the first sentence, but not with the second. Yes, governments might eventually put more care onto reducing covid counts. But a) it might be too late. Just check how long it took for travel restrictions to be imposed.

And b) the panic that people will be in will be even bigger, especially those who think that masks and vaccines are a way to spread microchips and 5G. These folks aren't rational.

Last, there is a non zero chance you'll be dying in the next few years. At that point, do you really care about rules? The governments will impose even stricter lockdown measures. But this will cause even more unrest as people are confined to their homes.

Ebola is in the category of "deadly but not easy to spread". Yes, it can be spread but by the time you spread it you physically can't leave your bed. That's a great help in preventing spread. I'm talking about a disease that at the start has few symptoms, allowing for easy spread, then gets severe and deadly further down in disease progression.


If COVID19 had been significantly deadly, presumably it would have burned out at the pace of SARS1.

It seems to be close to the sweet spot in terms of being able to spread but still occasionally kill.


If it was more deadly it would spread less quickly. That's how diseases work from my vague university memories of an epidemiology course. They are on a spectrum of deadly to contagious.


What actually causes this though? For example, take something like HIV that takes a long time to show symptoms, but have an airborne version of it. Do the things that make viruses airborne also keep them from having a long gestation period and also highly lethal?


> Do the things that make viruses airborne also keep them from having a long gestation period and also highly lethal?

Surviving airborne before the advent of buildings meant surviving outside in direct sunlight with little in the way of protection from droplets or anything else. Organisms have a limited amount of abstract "evolutionary capital" and the costs, so to speak, of developing adaptations to survive airborne and evade an active immune system are astronomical. It's not impossible, but the two "features" work against each other during the development phase.


(Not an immunologist/virologist/biologist of any stripe. The following is my rough layman's understanding of the answer to this question. Would love to be corrected by someone with more formal knowledge of the subject.)

My understanding is that the main reason is a biological application of the principle of parsimony: Evolutionarily, organisms don't generally evolve lots of different things all at once. Thus, if you have a novel variant of a virus, it is highly likely to be mostly like existing variants, but with a very small number of changes.

Basically, you can think of it as the virus having to hit a particular threshold of a mutation direction that increases transmissibility to become widely-spread, but also has to hit a particular threshold of a mutation direction that increases lethality in order to become very dangerous.

As soon as it hits that transmissibility threshold, that is the version of the virus that's going to spread, and hitting both those thresholds at the same time is going to be incredibly rare.

What's much more likely (and genuinely concerning) is the possibility that a virus that has already become highly transmissible evolves more in the "lethality" direction and goes from being a widespread nuisance to being a deadly pandemic. The longer we let something like COVID remain widespread, as I understand it, the more likely we are to see a variant emerge that retains its high transmissibility but also gains a higher lethality. However, as we can see from the fact that the bodies are not piling up in the streets, even after nearly a year and a half of a pandemic form of a particular virus, it's by no means guaranteed that something like that will happen, even at a small scale.


This is such an interesting question, I hope it doesn't get buried before someone knowledgeable answers it.

We all know the more aggressive a pathogen is, the more it paints itself into a corner because it's burning its bridges. Something like airborne HIV would be really scary.

My guess is that airborne diseases are not weaker, but that evolution has selected for resistance against those because they spread fast among the population.


This is unfortunately just not correct in real life. It’s a good representation of general behavior/trends, but for instance Smallpox was around since at least 500 BC, and still had a 35% fatality rate until it was eradicated in the 70s.

The more times you roll the dice, the more often you’ll get that magic combo of lethal and highly transmissible. For the most part it doesn’t favor it, but that just makes it an outlier when it does, not impossible.


At least a part of it is probably the human factor. The big "PR" problem we have with fighting the epidemic is that a lot of people won't have much (visible) problems. If COVID would lead to a slow and painful death 99% of the time, there were probably far fewer people trying to get it or using the herd immunity strategy.


I don't think we know what causes it. It is mainly just observed. What causes this is a very active area of study. Probably some output of a "evolutionary" process of how viruii mutate but my knowledge is very little :)


Sure, but this does not in any way favor prions over other options.


(Quick correction: PNG, not NZ.)


[flagged]


Ban... prions?

Do you think that you can go to the store and buy prions?

Maybe we can also ban the common cold and HIV while we're at it.


I believe that was sarcasm my friend.


Agreed. Prions almost strike me as an interesting boundary condition for stupid behavior. But if most of the world's meat supply becomes effected by prion caused diseases (think: Chronic Wasting Disease https://www.cdc.gov/prions/cwd/index.html ) then I think we truly might be in a tight spot.


IIRC, BSE (Mad Cow) was spreading among cattle because the industry was using meat-and-bone feed from infected cattle. It was tainting the meat supply because captive-bolt guns spray more brain around than more traditional hammer-oriented methods of slaughtering beeves.

So yeah, cannibalism and brain spray. Stupid behaviors.


Luckily humans can survive quite well without any meat at all.


I’m not sure this would save you. The deer get CWD by eating the grass other deer have been around. A zoonotic CWD would find its way into plant-based diets.


The article suggests it's transmissible through plants and even air. You can't even sterilize surgical equipment under normal protocol to kill them.

It is basically a juggernaut.


Agreed. What 2020 has shown us is that many humans can't cope with change well. This is what would cause us to be in a tight spot (IMHO)


If it was deadly enough, that problem would take care of itself quite quickly. Tragically, but quickly.


So can deer.


Unlike deer, at least some humans are capable of not pooping and drooling on other people's food.


ITYM affected, not effected.


Prions are pretty scary, but I think there are a lot of things this article gets wrong.

- Prions are resistant to sterilization (since there is nothing to "kill"), but simple soap and water are still highly effective

- Prions don't "last forever". They can live in buried carcasses for a few years, but some studies have shown that even our existing sewage treatment processes already break down a lot of prions.

- While vegans might have some risk, meat multiplies the risk because of how it can concentrate prions. I have to imagine that vegans have an incredibly low risk profile for prions.

- Most importantly, viruses and bacteria innately "want" to spread. Prions do not have any biological mechanism for their own propagation - they are just an unfortunate mistake of nature. And this seems to be key why there has never been a massive prion outbreak.

If you were an anarchist rooting for the collapse of society, as this writer seems to pitching for, sure it seems like it might be a more likely vector than many.


> Most importantly, viruses and bacteria innately "want" to spread. Prions do not have any biological mechanism for their own propagation - they are just an unfortunate mistake of nature. And this seems to be key why there has never been a massive prion outbreak.

I disagree here, and but it's not fatal to your argument. Anything that reliably reproduces "wants" to. Hell, I bet prions can evolve too on large enough time scales (heterogeneous induced refolding), so we can say they are alive. But sometimes the tortoise is too patient and the race ends before the hare tires.


The prions are not involved in the formation of new proteins, so it's very^100 difficult that they can evolve to create more efficient versions. [I try to not use "impossible" because life finds a way ...]

Virus instead use their own material as the original to make copies, so any new good or bad variation will be copied in the offspring, and they can evolve.


In order to evolve there has to be some kind of genetic information passed on to descendants. But for prions there is no such mechanism. A lower energy state is assumed and that's that.


There does not have to be genetic information for evolution.

Evolution only requires "descent with modification". DNA is a mechanism for this, but it is not necessarily the only one.


And prions don't have descent with modification. It's a regular mammal brain protein that got accidentally constructed backwards. There's nothing to pass on, nothing to modify.


Err, I think I remember a couple pretty famous articles that contradicts that prions generally don't have descent with modification, which may be what the person above us is referring to:

https://pubmed.ncbi.nlm.nih.gov/20044542/

https://pubmed.ncbi.nlm.nih.gov/17142317/


That's really neat. Still there should be a limited number of possible variants right? Like the energy landscape is fixed and will only admit so many possibilities?


FWIW Wikipedia says:

> Prion replication is subject to epimutation and natural selection just as for other forms of replication, and their structure varies slightly between species.[19]

[19] is https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2848070


descent without modification sounds pretty hard to achieve for all but the simplest things.


This got me wondering: what is the advantage of genetic information (DNA / RNA) over a self-replicating entity without those convenient molecules?

My impression is that DNA / RNA-based organisms have a high tolerance for changes to their DNA / RNA, such that many genetic changes won't cause them to lose their self-replicating ability. This is great for evolution.

Prions, on the other hand, seem like they would have a low reproductive tolerance for changes to their structure.


So prions are simply a result of entropy? Am I understanding that correctly?


All evolutionary change (and life itself) is due a combination of differences of entropy (aka an energy gradient existing), combined with randomness in the current environment allowing mutation/change and hence selection pressure.


I would say life is an artifact of non-erodicity. Rare things that self-replicate are over-abundant in hindsight.


> I have to imagine that vegans have an incredibly low risk profile for prions.

What do you reckon the risk profile of vegetarians would be? Are prions likely to end up in eggs and milk?


In general I think we should be trying to eat foods that are really biologically dissimilar to humans to avoid being sickened by our foods' diseases. Plants and fungi don't seem to cause pandemics. Mammals do (SARS, covid, swine flu, possibly Spanish flu). Birds can make us sick (salmonella). Fish, mollusks, insects, probably safer, parasites aside.


Does your second point suggest that we should avoid eating beef?


I think there's better arguments than a risk of prion disease for cutting beef (or meat in general) from your diet - not sure if your comment is meant as a joke that I'm not catching, so I'll hold out on going into details unless you're interested :-)


Not meant as joke. I'm aware that there are lots of good reasons for not eating beef, I was wondering if prion exposure risk is one of them, as the meat/no-meat argument is one I try to revaluate regularly as new info comes to light. I eat beef once or twice a week as I have found it hard to get sufficient iron, protein, b12 and enough calories in general without it (and other meats). That and it tastes good, but I'm always receptive to new counterpoints.


Prion concentration varies greatly from one part of a cow to another, because prions gravitate toward nerve tissue for some reason. The brain is definitely dangerous to eat. So are the eyes, the spinal cord, and parts of the intestines. Muscle tissue on the other hand is said to be much less dangerous. I don't know how much less, though.

Remember that all risks are relative. Just like radiation, there's likely to be a background level of prions in the soil that you can't avoid even if you only eat vegetables. The question is how much prion exposure you can tolerate without having noticeable symptoms within your lifetime.


I don't think prion disease currently is a significant enough risk to make it an argument against eating meat. It's a rounding error compared to for instance increase in colon cancer

I eat meat occasionally as well. I tried going vegetarian for a six months, but I lost an unhealthy amount of weight (my BMI dropped from 19.2 to 17.8) and I think the health risk from malnutrition, as you also mention having struggled with, is much more severe than that of prion disease


Yeah this is exactly the situation I find myself in. I regret the ethical and environmental consequences of eating meat, but I found it very hard to replace it completely and still have a balanced diet.


I take a liquid supplement called Floradix for b12/iron. It is made from yeast and vegetables. I'm prone to anemia and it has been immensely helpful.


I mean, there are a lot of reasons to not to. And I say that as a meat eater.


For people who are intensely worried about prions, it seems that eating less meat will decrease their chance of exposure.


So one of the oldest known prion diseases is a disease of sheep called scrapie. It is not transmissible to humans, thankfully.

It turns out that there is genetic resistance to it. There's a specific gene, of which there are two forms, called Q and R. If a sheep is Q-Q, it has no resistance. If it is Q-R is has some resistance. If it is R-R it is completely immune.

All of which is to say: we should not just assume that exposure to prions results in a 100% disease rate, nor should we assume that prion disease itself has a 100% mortality rate. Because the diagnosis for prion disease is clinical. In other words, we only know someone has the prion if they show signs of the disease. And we only look for the disease once someone starts showing significant signs of disease. But there is no test that shows that you ave been exposed, nor is there a test that shows you have been infected but that your infection is spreading so slowly that (say) prostate cancer or heart disease will kill you long before the prion does.

https://en.wikipedia.org/wiki/Scrapie


prion infections are always eventually fatal, there is no cure, and they are contagious.

The majority of research into prions happens post mortem, and is usually trying to pin down the cause of death. Statistically, there must be prions that are not lethal.

I'm reminded of the issues with people that work at pig processing plants, who had the job of blowing the brains out of the skull cavity with compressed air. Many were not issued proper PPE and ended up with a wide variety of symptoms.

https://www.twincities.com/2008/02/06/minn-to-question-25-mo...


I think that's an interesting point. Do you have a source for your statement, that most of the research happens post mortem?

It makes sense to me that non-lethal cases wouldn't be discovered, as it's probably prohibitively expensive to screen for non-lethal/dormant infections. It could also be that the progression is so slow for most people, that they die from unrelated causes before the disease becomes a problem (this is pure conjecture on my part)


The first promising blood test for mad cow (vCJD) was developed in 2016. Prior to that, "The only current method to diagnose vCJD is to perform a biopsy or a postmortem analysis of brain tissue." (NIH 2017).

https://www.nih.gov/news-events/nih-research-matters/new-met...

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5538786/

Scientists have since developed a promising method that relies on analyzing the retina.

https://www.nih.gov/news-events/news-releases/scientists-ide...


Thank you for the links - I look forward to reading the articles :-)


I remember that in the 90s, when "mad cow disease" was dominating the headlines, it was predicted that there would be an exponential increase in cases, decades down the line. The same point about prions being virtually indestructible was being made, the same concerns about surgical equipment and blood donations were raised.

Suffice it to say, the prion disaster did not materialize, so I'm skeptical about the same story being repeated almost verbatim today. My guess is that prions aren't all that contagious after all, especially after basic food preparation measures.


The concerns about blood donations continue to this day. A few years ago I went to a blood bank event not even thinking about vCJD and was turned away because I happened to live in Europe for more than 6 months in the early 90s.

It is a bit unnerving to know you may or may not have a disease that there is no test for, and symptoms may not appear until decades later.


You might want to check again to see if you’re eligible now. They ended some of these restrictions on blood donation in the summer of 2020.


Thanks! It appears that the new restrictions no longer apply to me. I’ll be off to give blood later this month.


...out of "an abundance of caution".

> It is a bit unnerving to know you may or may not have a disease that there is no test for, and symptoms may not appear until decades later.

That's true for many neurodegenerative diseases. If that's unnerving to you, better stay away from WebMD...


Who knows. I'll give you an interesting thought.

There is NO single, conclusive test for Alzheimer's disease. The symptoms are almost identical to CJD (human mad cow) with the biggest differentiator being time from diagnosis to death. Even Amyloid-beta plaque buildup is often (though not always) noted in CJD patients [0][2]. Likewise the other Alzheimer's marker tau protein is also elevated in CJD patients [3]. I'd note that we don't actually know that CJD must progress over 1-2 years rather than a decade -- it is an observational assumption.

Between 15-30% of Alzheimer's diagnosed patients also don't have the normal Alzheimer's brain symptom (presumably amyloid buildup) either. Alzheimer's as cause of death was 17.6% in 2000, but is now 37.3% in 2020.[1] Because CJD is defined as killing quickly, it isn't even checked for in such cases (a kind of self-fulfilling prophecy if the diagnosis criteria aren't actually accurate). It doesn't help that cleanup of a contaminated area is time consuming and costly (not to mention potential negative press and panic).

This isn't a new idea, but perhaps those fears were somewhat true, but have been buried under our lack of knowledge of protein diseases.

[0] https://pubmed.ncbi.nlm.nih.gov/3092727/

[1] https://alz-journals.onlinelibrary.wiley.com/doi/full/10.100...

[2] https://pubmed.ncbi.nlm.nih.gov/12891683/

[3] https://pubmed.ncbi.nlm.nih.gov/20881758/


> The symptoms are almost identical to CJD (human mad cow) with the biggest differentiator being time from diagnosis to death.

Ordinary CJD has a much lower median age of death (68 years) than Alzheimer's (88 years), but vCJD (the form that is believed to be caused by contaminated food) has a median age of death of only 28 years.

https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_dise...

A surge in young or middle-aged dementia cases would likely not have fallen under the radar.

> Between 15-30% of Alzheimer's diagnosed patients also don't have the normal Alzheimer's brain symptom (presumably amyloid buildup) either. Alzheimer's as cause of death was 17.6% in 2000, but is now 37.3% in 2020.[1]

You have mistaken the rate of deaths (per 100,000) for the percentage. When judging this increase, one must consider that in the same timeframe, the mean age of the population has risen by 10% and life expectancy increased by more than two years.


> You have mistaken the rate of deaths (per 100,000) for the percentage. When judging this increase, one must consider that in the same timeframe, the mean age of the population has risen by 10% and life expectancy increased by more than two years.

Sorry, knew what I meant, but messed up what I typed. In any case, doubling the death rate per 100k is still much greater than the 10% age and 2-3% lifespan increase would indicate. Alzheimer's mortality had increased 16x between 79 and 91 (from a mere 857 to 13,768 in 1991[0]. The previous study I quoted above put the 2018 mortality at 122,019. Alzheimer's is now the 6th leading cause of death and continues to rise at a rapid rate.

> Ordinary CJD has a much lower median age of death (68 years) than Alzheimer's (88 years), but vCJD (the form that is believed to be caused by contaminated food) has a median age of death of only 28 years.

This is the crux of the problem.

We have experimental knowledge about this from kuru. The last patient displayed symptomatic kuru some 5 decades after the cannibalistic practices had stopped.

Do only young people eat meat? If all age groups eat meat at equivalent rates and infection rates in cattle are constant (believed to be around 1 in a million IIRC), then the median age should be the population median age PLUS a shift for however long it takes to become symptomatic.

Linkage is also important. How do we know that CJD and vCJD are different? We made that distinction arbitrarily and don't have evidence that "normal" CJD is not caused by external sources. Official vCJD cases are around 200 over the past 30 years, so there isn't exactly huge amounts of data to pull from either.

If CJD really did explode in the 80s-90s, then you would expect the older generation to become symptomatic at an older age than would be typical if the disease were common in the decades before.

There seems to be a connection with being vegetarian/vegan and alzheimer's but how much is due to not eating meat and how much is due to overall healthy lifestyle is hard to determine.

Something weird is going on with Alzheimer's disease and the legit explosion in cases over the last 30 years (both per capita and as cause of death in the elderly) give reason to wonder what weirdness is going on.

[0] https://www.cdc.gov/nchs/data/series/sr_20/sr20_028.pdf


Here is another major difference:

"We know that the β-A4 amyloid of Alzheimer’s disease also derives from a normal host protein that in diseased people accumulates in the brain, but it does not have the ability to transmit disease to a healthy person. Why this difference?"

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1114482/


We have no idea if US Beef is infected. The USDA prohibits anyone from testing their cows and ended their spot-testing program (which was a joke).

So unlike Europe we never reckoned with the problem and don't know if it is currently spreading. For human exposure we would not yet be far enough along to know if the US Beef supply either is or was contaminated.

Personally I have basically stopped eating beef for this reason.


It is estimated that hundreds of thousands of infected cattle entered the food chain in the 1980s. The total number of cases of vCJD is at less than 250 as of 2018.

Feeding practices that led to BSE have changed, the USDA is still testing for BSE, and if the disease was spreading, we'd probably notice it at some point.

Personally, I'd be far more worried about cattle farming practices spawning some sort of super-resistant flesh-eating bacteria than anything related to BSE.


Also worth noting that only four of those cases occurred in the US, and none of those have been traced to US beef; in each of these cases, the patients had spent a significant time living outside of the US (and two of the patients lived in the UK, whose cows were known for having BSE).

https://www.cdc.gov/prions/vcjd/vcjd-reported.html


>The USDA prohibits anyone from testing their cows

Huh? Why would they prohibit testing?


The USDA does test a sample of cows, but prohibits companies from doing testing on their own cows probably because the tests would likely be inaccurate and provide an unwarranted impression of safety:

> NOT A FOOD SAFETY TEST

> BSE tests are not conducted on cuts of meat, but involve taking samples from the brain of a dead animal to see if the infectious agent is present. We know that the earliest point at which current tests can accurately detect BSE is 2-to-3 months before the animal begins to show symptoms. The time between initial infection and the appearance of symptoms is about 5 years. Since most cattle that go to slaughter in the United States are both young and clinically normal, testing all slaughter cattle for BSE might offer misleading assurances of safety to the public.

> ...

> Why doesn't USDA test every animal at slaughter?

> There is currently no test to detect the disease in a live animal. BSE is confirmed by taking samples from the brain of an animal and testing to see if the infectious agent - the abnormal form of the prion protein - is present. The earliest point at which current tests can accurately detect BSE is 2 to 3 months before the animal begins to show symptoms, and the time between initial infection and the appearance of symptoms is about 5 years. Therefore, there is a long period of time during which current tests would not be able to detect the disease in an infected animal.

> Since most cattle are slaughtered in the United States at a young age, they are in that period where tests would not be able to detect the disease if present. Testing all slaughter cattle for BSE could produce an exceedingly high rate of false negative test results and offer misleading assurances of the presence or absence of disease.

> Simply put, the most effective way to detect BSE is not to test all animals, which could lead to false security, but to test those animals most likely to have the disease, which is the basis of USDA's current program.

The ban might not be warranted (I don't believe it is myself), but it is important to be aware that testing for BSE may not be accurate at the time most cows are slaughtered.

https://www.usda.gov/topics/animals/bse-surveillance-informa...


Because businesses don’t want to bear the loss (financial and likely reputational) of finding out that their beef contains prions.


Thereby combining all of the potential isolated, individually bad disasters into one enormous fully-correlated watershed moment when all companies are simultaneously found to be selling prion-containing beef...


If US beef was contaminated, wouldn’t there be thousands of cases of vCJD[1] by now?

1. https://en.wikipedia.org/wiki/Variant_Creutzfeldt–Jakob_dise...


Yes there could be. It remains latent for years after consuming the infected meat. Guess we'll find out! ¯\_(ツ)_/¯


We did find out. The US has had a total of 4 cases of vCJD, all of which were contracted overseas.[1] For comparison, the UK has had over 170 cases.[2]

1. https://www.cdc.gov/prions/vcjd/vcjd-reported.html

2. See table 3 in the PDF on this page: https://www.termedia.pl/Review-article-r-n-r-nVariant-Creutz...


It isn't really possible to prepare beef in a way to eliminate prions; you have to just make sure the beef you're preparing doesn't have them.


I'm aware that prions are hard to destroy, but that's a different question as to whether they are contagious. A lot of contaminated beef did enter the food chain, yet the expected surge in vCJD did not occur. I would expect cooking to have some impact here, but that's just a guess.


> Ancient and odd but relatively easy to talk about, viruses are the perfect vector for the horrors peddled by the BBC, New York Times, and their ilk.

Proceeds to peddle the "horrors" of prions.


Yeah I'm not a huge fan of the way the article is written. The concerns are undoubtedly real, but the pearl-clutchy tone and the endless barrage of asides and pop culture references make me doubt the author's conclusions.


This guy is trying really, really hard, wildly inflating our susceptibility, our risk, the vectors by which prions can be transmitted, everything. I hope I never sit next to him on a long plane flight.


That can be generalized to any journalist or media organization. Their currency is fear.


What reputable journalist or media organization claims that the secret jewish new world order is using vaccines to inject people with prion diseases like this guy does?


I for one would appreciate anyone who has expert biological knowledge commenting on this.

I don't entirely dismiss this article since I have a friend who worked with prions in a bio lab at the Univ. of Chicago and I remember he was absolutely terrified of them. (And IIRC he reported having some doubts about laboratory safety protocols...) But I don't think he was afraid of this kind of apocalyptic endgame either; he just thought they were horrendously nasty stuff.


The limited research there is on prions is heavily concentrated on the nasties. Its like learning about bacteria by studying Mycobacterium tuberculosis and coming to the conclusion that bacteria = death.


>The anti-globalist movement of the 90s is long dead. The far-left which fueled it has been co-opted, down to the level of its language and internalized identities, by global business interests. Coordinators of anarchist riots appear in Forbes; there are anarchist professors and journalists.

No citations

Lots of fear

Thinly veiled far right rhetoric


This article is coming from the far left, not right. I think he is bemoaning the lack of real anarchists.

But perhaps horseshoe theory strikes again.


I understand the argument here, and how ill-prepared we are for steady low-coefficient exponentials, but never more have I felt like the 2nd law of thermodynamics is my friend.

Even if 1 prion can kill you, even if prions will survive in the soil indefinitely, I think we are going to erode all the topsoil faster than the prions can accumulate in concentrations sufficient to cause issues.


As I read this, cellular automata lurked in the back of my mind. Despite the stochastic details, there is an eerie clockwork marching hiding under the covers of life.


When you put it that way, it sounds oddly poetic.


> Prions Are Going to End the World

Get in line.

/* reads TFA about prions binding in soil */

Please move to the front of the line.


Prions used to bug me as an idea for quite a while when I was an undergrad. However, since I have worked in a biolab and I actually read material that isn't just fear mongering, I can tell you a ton of stuff that makes this article totally wrong.

Prions don't live forever, they are susceptible to soap and water. Prions are proteins, anything that can break down a protein will work to break down a prion. They are not some form of mystical protein. They will break down naturally over time. This is why regions that have had Kuru outbreaks have been able to stop those outbreaks.

Since prions are literally just a chemical (albeit a complex one), they are not trying to propagate themselves. There have been outbreaks, but no massive ones with prions because that's simply not how they work. As well, since they are proteins and proteins eventually break down when they are removed from a living being, they will also eventually break down.

This article is a whole lotta fear with not a whole lotta valid info.


The article is completely wrong about the prion mechanism.

>The prion’s structure is corruptive because it is not a copy but a mirror image of the protein, flipped in the opposite direction. In technical terms, some groups of prions have the opposite chirality—Greek for “handedness”—of their normal cousin proteins. The prion is doubly corruptive because it does not keep its inversion to itself: when it encounters its mirror image—a normal protein—it convinces that image to flip, to change chiralities and join the prion group.

The amino acids don't change their chirality, only the protein's secondary structure.


So, is it because prions managed to attain a lower energy configuration than similar proteins, forcing them to reconfigure themselves upon contact with a prion, causing a cascade of protein deformation that is super stable and can't be reversed? Like protein configuration = local minimum, prion configuration = global minimum?


It's like the protein version of vacuum collapse.


Prions are misfolded proteins. Because of mutations our proteins have tiny variations in them and it should not surprise me if some of them are more prone to misfolding than others. Currently it barely matters, but in a world rampant with prions, wouldn't we evolve more stable ones?


There's some space for prion evolution, but it's a very small set of possibilities. And more importantly, the little they can evolve, they do so at random, there is no genetic recombination step to merge the selection of unrelated features.


You misread me.


Oh, indeed I misread it.


Evolution doesn't do magic.

You could just as well ask, "in a world with solar UV rays, wouldn't we evolve cancer resistance?"


Arguably, we did. Our scalp is protected by hair, our flesh is covered by a skin, and the skin can tan. Then there are dna repair mechanisms, and apoptosis as the final line of defence.

Cancer mainly takes the old, and is certainly no threat to the survival of mankind.


We have evolved UV ray resistance, it's called being black.


Prions are like the real-life version of ice-nine. Just much slower moving.


Why do prions cause our proteins to reverse, and not vice versa?


Asking naively: is anybody doing research on good prions: proteins that get other proteins to fold correctly/helpfully, beating out bad prions in the process?


You might be interested in reading about "chaperone proteins"!


Why this get flagged?


If the issue is a protein folding the wrong way, and inducing that fold into nearby proteins, why doesn't this just happen spontaneously in humans?


I came across an article about vaccination against ingested prions a while back.

I couldn't find the exact one that I read a year ago... the one I read talked about a test where the vaccinated mice survived while the unvaccinated mice died quickly.

Prion diseases are scary psychologically because the early stages are almost the same as any other neurodegenerative disease. For those who are suffering from one, who would likely develop some sort of depression early on, really gives them doubts about their lifespan. This is from personal experience.

Here's a bunch of articles about it:

https://pubmed.ncbi.nlm.nih.gov/18215090/

https://pubmed.ncbi.nlm.nih.gov/17234125/

https://www.eurekalert.org/pub_releases/2014-12/nlmc-fsv1217...


I wouldn't be too worried about this. I'm noticing chatter in the worst parts of the internet around COVID vaccines causing prion disease, it's likely just the latest disinfo by Q types.

This site hosts white-supremacist aligned articles (like this one supporting the dehumanizing NPC meme: https://www.countere.com/home/how-to-spot-an-npc) so I wouldn't be too worried about an article here about the "end of the world"


Ice-nine is the real threat!


TLDR: "prions are ice-nine for proteins across all life"


I’m surprised these haven’t been weaponized by terrorists.


Too long between "attack" and result? Most terrorists/freedom fighters (pov required) seem to be attempting to further their own causes.

Wouldn't this be more for the lunatic who either hopes to take humanity down in the long run, or just does not give an f?

More of a 12 Monkeys scenario?

Regardless, happy Monday, indeed...


I think the timescale is too long for it to be an effective tool for terrorists

Terrorism is first and foremost a propaganda strategy to destabilise the political system in the target country and recruit new members to the organisations cause

Since prions take decades to progress, a terrorist attack using such a weapon, would probably not create the shock and outrage, that makes headlines


It would be the perfect timetable for an adversarial country looking to become the next superpower to use as a bio weapon though.


What would the delivery system be? Waterborne? Food-borne?


(I read the OP comment as sarcasm.)


Also sarcasm


Whoosh! (me, LOL)


It's okay. Lack of sarcasm tells in textual format leads to Poe's Law. ;) I wish English had inline accessory indicators to tack on tone for context instead of ambiguous wording, especially for critical conversations (that probably should be had by phone or in-person). Perhaps emoji serve this purpose? Why not some sort of diacritics though?


[flagged]


Read the website of the author of that garbage paper and decide for yourself whether you feel like it’s a good decision to broadcast it to a wider audience:

vaccines.net

> We are concerned that the current outbreak of COVID-19 is actually a bioweapon attack and may be linked to the US anthrax attack of 2001, which originated from the US army base Fort Detrick.

> The former FBI agent in charge of the US anthrax attack investigation, Richard Lambert, alleged that his superiors acted to inhibit his investigation and prematurely closed the case after it was determined the anthrax used for the attack came from Fort Detrick. This revelation (see this link) as well as Dr. Classen's first hand experience working 3 years at Dr. Tony Fauci's NIAID leads him to be more than concerned that there is a contingent in the US government that is not acting in the public interest regarding bioweapons. Classen notes NIAID received substantial funds for bioweapons research after the domestic anthrax attack . He considers this quite alarming given activities he observed at NIAID and the relationship he observed between people at NIAID and the nearby Fort Detrick.


For additional context, the author is JB Classen. From his Wikipedia page:

> A widely-reposted 2021 Facebook post claiming that the mRNA vaccines against COVID-19 could cause prion diseases was based on a paper by Classen. The paper was published in Microbiology and Infectious Diseases, whose publisher, Scivision Publishers, is included in Beall's list of publishers of predatory journals. Vincent Racaniello, professor of microbiology and immunology at Columbia University, described the claim as "completely wrong".

https://en.wikipedia.org/wiki/J._Bart_Classen


A more thorough dismantling of the scientific claims in that paper in case anyone is offended by not addressing the writings of a moron on their merits:

https://sciencebasedmedicine.org/can-mrna-based-covid-19-vac...


> Ah yes, HN: we downvote science we don’t like.

Calling something science doesn't make it so.


The abstract caught my attention and so I kept reading. The further I read, the less reputable the article came across.

The following quote from that paper reads a lot like typical anti-vax garbage:

“Many have raised the warning that the current epidemic of COVID-19 is actually the result of an bioweapons attack released in part by individuals in the United States government [10,11]. Such a theory is not far fetched given that the 2001 anthrax attack in the US originated at Fort Detrick, a US army bioweapon facility. Because the FBI’s anthrax investigation was closed against the advice of the lead FBI agent in the case, there are likely conspirators still working in the US government. In such a scenario the primary focus of stopping a bioweapons attack must be to apprehend the conspirators or the attacks will never cease. Approving a vaccine, utilizing novel RNA technology without extensive testing is extremely dangerous. The vaccine could be a bioweapon and even more dangerous than the original infection.”



I've dug around into this, and I'm certainly no more than an armchair biologist, but it seems highly unlikely to be a real risk. If spike proteins could mis-fold and become self-reproducing prions, this would have happened by now "in the wild" and it wouldn't depend on an mRNA vaccine to trigger the problem.


I stopped reading at “vaccines cause diabetes”.


Could this explain why everyone seems to be getting dumber? Have we all been infected by prions already and our brains are decaying?


I wonder if there might not be something similar to the Prion mechanism that cause the healthy brain protein to “change chiralities” and join the prion group on the atomic level that might give insight into the matter-antimatter asymmetry problem.




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