French moratorium on prion studies after two cases of fatal brain disease (2021)
science.org
science.org
However, your chances of getting prions is very low. As long as you avoid "exotic" meat like monkey brains and stay in countries with strong regulatory bodies (like the US/EU etc) the risk of exposure is extremely limited. Bonus points if you avoid swimming in random bodies of water to not get contaminated water with brain-eating amoeba traveling up your nose.
I've always wondered if the case had merit and we just did not have sufficient medical knowledge/techniques at the time to investigate the source, of if someone read about prions and self-diagnosed.
https://www.austindailyherald.com/2009/02/hormel-qpp-lawsuit...
Wow! Thanks for one of the most disgusting imaginations I had in a while! :-)
I thought those were thought to be autoimmune issues from the person's body learning to treat neurons as a threat? Or is that a separate thing? (Or outright discredited?)
As noted in the article, prions are also responsible for 'mad cow disease', Bovine Spongiform Encephalopathy (BSE) as well as Creutzfeldt–Jakob disease. If you really want to get into prions you can start with the wikipedia entries for those. They're pretty incredible from a physics / biology standpoint.
The thing the article doesn't point out that is important to keep in mind is that CJD is a really awful neurological wasting disease and it's likely that in those seven years Émilie Jaumain and her family suffered terribly.
and scrapie in sheep, and Chronic Wasting Disease in deer and other wild Cervidae in North America. The latter is increasingly common in the US, although its wide spread is recent enough that little is known as to whether it is being transmitted back to humans through hunting and processing of deer.
Anyone care to modify my plot to make it spicier?
I'm assuming that bleach is insufficient for this purpose.
I'm sure I'm not particularly unusual in this respect. Most of friends have similar preferences.
South-East Asia is a hugely popular destination. Do we know different demographics?
Apparently 42% of Americans have passports now, but it was about half that just a decade ago, and I'd attribute most of the growth to immigration, not vacations. Anecdotal, I'd say in SEAsia the "foreigners" I encountered were like 80% European. In Central America, the cheapest/shortest flight from the US, it was still like 50% Europeans. Whenever we're finally able to visit my wife's family again, she's goin to have to get special permission to take unpaid vacation time or else change jobs in order to be get enough time to make the trip to Asia worthwhile. That's pretty typical here.
[1] https://www.cbsnews.com/news/woman-dies-from-brain-eating-am...
We do have a fair amount of people who go to Mexico, but even then, It's extremely common to not go.... anywhere, unless you have money, and especially not go anywhere regularly, mostly because it's expensive and nobody can afford time off work even if they could afford plane tickets.
It's semi-possible, but as far as I can tell lot of people don't really save up and make it a priority.
This has all gone a bit off track. To bring this back to the original context:
> Doing that sort of travel is, for most citizens of the industrialized west, pretty unusual.
"Pretty unusual" is what I'm disputing. I'd say it's far from "pretty unusual" for average, regular people to go on holiday outside Europe.
"Strange women lyin' in ponds distributin' swords is no basis for a system of government!"
But the comment that I was responding to was "Doing that sort of travel is, for most citizens of the industrialized west, pretty unusual." which seems to be more about "outside of Europe" than it was "and swimming in freshwater. That was the part I found strange. I think there's a couple of countries with much lower than average travel (the US already mentioned and I seem to recall that the French tend to stay within their own borders more than the average)
Especially when you see micrographs of the little demon: https://www.newsweek.com/brain-eating-amoebae-strike-again-t...
After all you almost certainly put them at more risk just driving them there.
I didn't say they stopped swimming, but it just added some bullshit on top that I would have rather done without.
Lots of Westerners travel to Asia/Africa/middle East/Latin America. (And yeah, sometimes they catch diseases).
Also, you have to think whether people who normally live in those countries (non westerners) get exposed and face the consequences regularly.
I wonder if this whole disagreement is actually about the fact that Americans tend to travel abroad less than those from other Western countries.
https://www.cdc.gov/prions/cjd/occurrence-transmission.html?...
Here’s some basic info and radiology https://radiopaedia.org/articles/creutzfeldt-jakob-disease?l...
https://www.merckmanuals.com/home/brain,-spinal-cord,-and-ne...
So scary.
Also, be very careful with the water that you use in a neti pot.
A radical (barbaric) way might be to immediately tourniquet the finger, anesthetize, and cut off the tip! They could hang a little guillotine next to the eye-wash station....
I mean… probably, but you’d need a bigger guillotine. And now having a hand sized guillotine in the lab is a bigger safety hazard.
Come on now, it's not like it would be a giant curved blade hanging freely from the ceiling into middle of the room.
"A 5-minute soak in a 40% solution of household bleach decontaminated stainless steel wires coated with chronic wasting disease (CWD) prions, according to a new study by National Institutes of Health scientists."
Although it notes: "Notably, the study failed to find an effective method to decontaminate CWD-infected solid tissue. Pieces of CWD-infected brain retained prion activity even after a 30-minute soak in 100% bleach. Investigators note that bleach fails to penetrate tissues and should be used only as a surface decontaminant."
There’s this case that my PI showed me of a student who pricked his finger with a needle filled with DCM [1] (which is very common solvent in used in lab). The residual DCM at the tip of the needle caused the damage shown in the pictures.
When your work involves handling tens of needles daily, accidents are bound to happen. I pricked myself once with a needle. Thankfully it was brand new and dry, so nothing happened. It was just the idea of it was terrifying. In an alternate world, I could’ve gone to the ER because of it. Hope someone invents a safer needle.
[1]: https://www.chemistryworld.com/news/gruesome-accident-prompt...
I drenched myself in DCM and stick myself with needles a few times.
Not to say the lab isn’t dangerous. I knew one person who peppered their abdomen with glass when a perchlorate exploded, scarred their face when a peroxide exploded and burned themselves with an organolithium.
But a simple needle stick with DCM residue won’t do that.
There are also gloves that protect against puncture. If you're using both sharp needles and non-protecting gloves then it is probably time to revise the procedures.
She discovered she has the gene for fatal familial insomnia when her mother died of it. She and her husband retrained to be biologists and now do cutting edge prion research. They have made some pretty substantial progress towards both better understanding and tracking of prion disease and also potential therapy.
https://www.genengnews.com/news/potential-prion-disease-trea...
What is the reason that we can thank for prion diseases not being much more common?
Another way to look at it is that the combined lifespans of the organisms of a food chain are shorter than the time it would take for one mis-folded protein to be likely to occur and then also cascade to a degree that would affect the health of the top organism. Prion diseases only show up when you introduce a loop in the food chain.
One reason is that we don't eat each other which would spread certain prion diseases like https://en.m.wikipedia.org/wiki/Kuru_(disease)
They don’t “want” to exist, even to the minimal extent of a virus, they just happen.
Prions are in that sense no different - they are just less successful.
I don't believe they reproduce in any but the broadest sense either. Don't they just cause other proteins of the same type to misfold as well? It's not really a transfer of genetic material, it's a change in the emergent structure of the protein.
The prion's mode of operation isn't just "bump into stuff". It reaches the brain, causes other materials (DNA aptamers) to clump together, and causes other prions to be created.
That one happens to have a part that looks like genetic material and the other looks like a misfolded protein is merely a small implementation detail to me.
(Disclaimer: I, like everyone else I'm this thread, is not a qualified source of medical information.)
I've never heard of prions targeting certain tissues. I need to do some reading, as it's been a while since my last bio course. Do you have an article that explains how that works? In order to have that ability, they would have to be more complicated than I understand them to be.
The difference is that viruses have many more tools available in order to evolve and adapt. Prions, like crystal polymorphs, merely have the configuration space of the molecule. Viruses have the configuration space of dozens of proteins, and complexity can increase, so in theory there is no upper bound.
So I guess yes, they are all replicators that differ in extent by the amount of phase space available to their lifecycle. But the complexity jump between prions and viruses is huge, and prions are inherently bounded.
One main "behavior" difference between crystals, prions and viruses is that a crystal only forms by recruiting its equals when in high concentration - growth, but no replication.
Most virus is way more complicated than prions as you say, but a blue whale is also more complicated than a single-celled myxozoa and yet they are both animals. I do not think a comparison of complexity is meaningful in this debate.
One should also consider that there are countless proteins to misfold in any number of ways, so the prion space is not necessarily that limited. Virus do have the upper hand by using our well-oiled evolutionary machinery though.
Viruses that don’t “want” reproduction fail to do so and disappear.
The viruses we see are common because they reproduce, it's not that they reproduce because they have some drive to become common.
Thus prions are less common because they aren't as successful in spreading (for some reason(s) I don't know either).
The essential idea is roughly "we end up with more of the things that replicate more". Despite the redundancy, there is a crucial idea in here. If we allow the use of the word "want" metaphorically then anything that replicates "wants" to reproduce including memes, crystals, prions and computer viruses.
Maybe you're just saying that a prion isn't as good at replicating as a virus? It seems like a difference of degree rather than kind.
They are qualitatively different.
A virus infects a cell, and critically, can quickly adapt (through random mutations) in an unthinking war between the various forces of the immune system and transmission mode efficacy; A prion does not evolve, it's a misfold of a single type of protein. If you want to compare it to a virus, it's like a virus that is incapable of replicating anything other than an exact copy - and can also only replicate itself using a particular cell (one type of protein).
Why the human body cannot effectively defend itself against particular propagating misfolded proteins is a mystery to me though... perhaps it's too low level to be identified by the immune system as something to attack, much like a poison.
That a virus can be a big and complicated structure does not mean that smaller and simpler structure is fundamentally different.
Viruses themselves also have many different modes of operation, with things like virophages, satellite virus and giant virus showing just how unclear the term "virus" is.
They are countable, but yes there are many ways, but that alone doesn't mean it's capable of meaningfully evolving. It's a search space multiple orders of magnitude smaller than a piece of DNA, so it's easy to see how a different successfully propagating misfold will be significantly less likely when starting from an existing propagating misfold.
If you are suggesting one propagating misfolded protein can, by chance, misfold into another propagating misfolded protein at a rate fast enough to become adaptive... then it's qualitatively the same as a virus, but I don't see this as likely given the significantly smaller search space.
The space of possible proteins - and thus prions - is not at all limited, neither figuratively nor literally. It is most certainly not "countable" without some Nobel-prize inducing discovery.
The only known human prion proteins seem to be a few orders of magnitude smaller than that though, and they don't appear to adapt.
Are you saying it's possible for prions to emerge from large proteins with the ability to adapt?
I guess what I am saying is that either you are right and that can be extrapolated all the way up the chain to humanity. Or that you are wrong and you can apply free will all the way down the chain.
In particular when you feed cows proteins made from dead cows it's making it more likely to create prion problems than when wild animals live in small herds in forests (and the ones that are sick are killed by predators or starvation before they can infect others more than likely).
So it doesn’t require it, it just increases the odds (and they don’t eat each other to spread it - it’s highly infectious and demonstratively infects if they share browsing/grazing land).
It doesn’t appear to spread between them and humans, but it definitely makes me nervous.
Today we have international animal shipping and trade, and global distribution. Prions are able to spread from exposure to shared pastures. A couple years of contamination used to be no big deal - today, if missed, it can spread all through the food supply.
The chance of any infectious agent "jumping the species" due to cramped conditions or because of exposure of workers to contaminated animal parts in unclean slaughterhouses is immense - the current SARS-CoV-2 pandemic in my opinion is a warning signal of something much worse to come, and no matter what kind of agent it will be, we're pretty much screwed. Half the population or more of the world doesn't believe in vaccination, a quarter or more don't have access to vaccines or any kind of modern medicine at all, we are already running out on reserve antibiotics...
Viruses rely on the machinery of nucleic acids to replicate. That machinery is complex and robust and the result of some long period of continuous evolution, which means viruses exists in a fairly smooth fitness space where mutation and recombination are effective means of improving fitness.
Prions by contrast seem more like isolated spikes in a much sparser fitness space. Presumably the equivalents of mutation and recombination for prions lead to molecules that almost never retain the capacity to replicate, so they have limited potential to become fitter over time.
Prions can't self replicate from raw resources: they depend on inducing conformational change in other proteins. The host has to produce, and use, the specific protein they can target in an accessible location.
The upshot of this for prions is that lacking genetic code, or a need for replication machinery, and being a variant of the protein they target, they are perfectly adapted for their environment and host and currently impossible to destroy without also damaging the normal correct protein form.
I’m far from knowledgeable in this area, but the model in my head is: the $foo_protein can be folded in at two important ways, $foo_v1 is what you want, $foo_v2 is a catalyst for refolding $foo_v1 into $foo_v2 but doesn’t refold much (or anything?) else.
A human analogy might be a disgruntled but charismatic worker who starts spreading a disruptive attitude until morale and productivity plummet in their department. As long as one of that team remains, the issue can still spread. Coincidentally, both the potted plants and highly compensated leadership are unaffected.
Not really on both parts. It's only transferred by eating meat so the defence is as soon as prion infection is detected all possibly infested livestock is culled and severe bans on trading are placed. Cannibalism in animal feed is also disallowed which makes transmission rare. Being vegetarian is also a complete defence.
They're also rare to appear, otherwise eating livestock would have regularly wiped out humans already. They're basically non-existent if you don't eat humans and livestock isn't fed to livestock.
Tell that to deer dying of CWD.
Your condescending sic is, ironically, misplaced.
Lady in the story could have been a Vegetarian yet still died from a Prion. As others have pointed out swimming in bodies of water is possible vector for prions.
But: consuming bush meat, brains, feeding dead animals to other animals, being careless with sharps and so on are all excellent ways to contract them.
We kinda take this for granted, but it may be the case that our deeply-rooted psychological disgust and aversion to eating human flesh is a product of prion disease killing populations of ancestors who did. Or perhaps, even our tendencies to ceremonies of death helped us: those that feared the bodies of the dead, or were more likely to create rituals of destruction and avoidance, were more likely to persist and pass on their genes :)
In addition, your body probably has evolved to use proteins that aren't particularly susceptible to misfolding AND accumulation. (Perhaps a long time ago, it WAS quite common?)
But why much more than a virus or bacteria? I am terrified of rabies, sure, but not in the same way. Something about prions rubs me in the weirdest of ways.
Maybe it has to do with them not being an "external" assailant (even if originally acquired externally), but an inherent defect of some part of myself?
I think my own fear of rabies is actually somewhat similar, but yours may be different.
Even Ebola with a microscopic amount of contact and immediate treatment you can recover.
Prions, not so much. Death sentence.
The only reliable prion disease diagnostic is brain tissue biopsy.
Articles make it sound like our entire being unwinds in their presence, while the real outcome is likely much less exciting: clumps slowly forming that fail to get cleared and end up causing problems as they accumulate. Fatal and no available cure once there are symptoms, but... Less scary.
(Disclaimer: I, like everyone else in this thread, is not a qualified source of medical information.)
The problem is that there are a minuscule number of prion deaths in the developed world, so I don't see it getting a huge amount of attention.
On the other hand, the Covid RNA vaccines were basically invented by a handful of people looking at a string of nucleotides over the course of five days. (It took large companies to actually develop the vaccine itself, of course.) This could end up being something that's solved by more accurate protein-folding simulations (perhaps Google's AlphaFold) and a couple of grad students.
If I suspect I've had contact with a meat or blood of a prion-infected animal, what could I do about it?
Prion diseases like mad cow kill within one year after 5-10 years of infection.
The massive wave of death and terror would end society as we know it
> Cattle are believed to have been infected from being fed meat and bone meal (MBM) that contained the remains of other cattle who spontaneously developed the disease or scrapie-infected sheep products. The outbreak increased throughout the United Kingdom due to the practice of feeding meat-and-bone meal to young calves of dairy cows
[1] https://en.wikipedia.org/wiki/Bovine_spongiform_encephalopat...
I know it’s obviously not that simple in a medical research environment, but I do wonder if the basic idea of a sewing thimble could be adapted into a contoured, finger-shaped thin metal layer that’s mounted inside special gloves for the researchers to wear. It would have to be done in such a way as to not hinder their dexterity, but I’d think that’s quite doable if said “thimble” was confined to the first segment of each finger.
> and did not wear both metal mesh and surgical gloves, as she was supposed to
Even the article itself doesn't seem to ask these questions.
It's especially dangerous with unscrupulous bosses, who will just shrug and not have a fight with their own employees over making them follow the regulations.
> she stabbed her left thumb with a curved forceps while cleaning a cryostat — a machine that can cut tissues at very low temperatures — that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE
Then later:
> Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. "We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans," Aguzzi says.
Am I missing something, or is the logical conclusion that Aguzzi thinks his lab is avoiding disaster by only using mouse-adapted sheep prions, but the lady is suspected to have been infected by an injury related to "transgenic mice infected with a sheep-adapted form of BSE". I hope there's some nuance here, because otherwise it sounds like Aguzzi's lab isn't actually avoiding infection. Perhaps the lady's case isn't considered to have definitely confirmed infectiousness due to uncertainty (though no uncertainty seemed to be mentioned)? Or the mouse-adapted sheep prions Aguzzi's lab uses are different to the transgenic mice infected with a sheep-adapted form of BSE? I have no idea; it's just that the apparent conflict between these two sections jumped out to my layman's eyes.
It immediately goes on to talk about their own discovery 10 years ago that prions could be spread through aerosols which "totally shocked" them and might "warrant re-thinking on prion biosafety guidelines" but that seems an entirely separate thing from the decision to use only sheep prions for research.
Previous discussion:
> In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice.
The HN headline:
> Woman dies seven years after pricking finger in prion research lab (science.org)
Where did the "seven" year quote come from?
Or were these experiments actually done in a BSL-2 lab?
Maybe I just think about cheesesteaks too much.
Why don't they just hire competent people?
We have BSL level 4 and people don't often prick themselves.
What gives?
Wuhan Institute of Virology was BSL level 4, and yet here we are.
People aren’t perfect. Accidents happen.
441 nuclear reactors have been operating flawlessly for over a decade.
Countless biology labs deal with dangerous pathogens daily with minimal accidents.
This French lab from the article had two separate accidents in three years.
I dislike when people use the term 'accidents happen' in a hand wavy way. There's a whole world of risk calculations to determine if something is safe or not.
Fascinating choice of timeframe: "over a decade".
How about going back just one more year. We can find multiple nuclear reactors blowing themselves up quite spectacularly on TV:
Over the following three weeks there was evidence of partial nuclear meltdowns in units 1, 2 and 3: visible explosions, suspected to be caused by hydrogen gas, in units 1 and 3; a suspected explosion in unit 2, that may have damaged the primary containment vessel; and a possible uncovering of the Spent fuel pools in Units 1, 3 and 4.
https://en.wikipedia.org/wiki/Fukushima_Daiichi_Nuclear_Powe...
Other than that, Mrs. Lincoln, how did you enjoy the play?
I never said that nuclear reactors don't have problems I would be an idiot to say that. Everyone on Earth knows nuclear reactors have problems.
Statistically though it's a very very unlikely.
What it sounded like you were saying is that because human error exists....we should shut down all nuclear reactors and biology labs.
I don't agree with that.