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France issues moratorium on prion research after fatal brain disease strikes

391 points by ta988 5y ago

ta988 5y ago
The article talks about the case of Émilie Jaumain that got contaminated by prion while cleaning a device used to slice mouse brains. She died 9 years later of a prion disease. After several cases of exposure across the world, France decided to block prion research for 3 months to try to study the issue.
- tinus_hn 5y ago
  They are going to stop studying so they can start studying the issue? Ah well, this is the level of ‘science’ we’ve come to expect in the pandemic.
  - Reventlov 5y ago
    No, they're gonna stop studying until they have reviewed all the security protocols regarding the handling of prions, and this, nationwide.
    - aqsalose 5y ago
      There are reviews and then there are reviews: Rubber stamp security reviews happen. I don't know much about this particular case, but I think, in general, a trustworthy review needs to be conducted by an independent body with genuine power (edit. and desire) to order things to halt permanently if they can not be made safe enough (and then there is a separate question of who makes the call "what is safe enough").
      - bluGill 5y ago
        >(and then there is a separate question of who makes the call "what is safe enough"). This is hard. If there isn't the right incentive they can decide nothing is safe enough. This ensures safety now, but it means that if a cure is possible it won't be discovered (or anything else useful from the research). The FDA has been accused of needlessly holding up useful drugs on this grounds (I'm not going to comment on if true or not)
    - antattack 5y ago
      They want to make sure that disease did not come from other places than the lab, which would be a concern to the general public. However unlikely, it's a possibility that has to be eliminated.
  - me_me_me 5y ago
    Yes, they should stop potentially lethal research procedures to see how they can prevent them. Its not rocket science.
    - pc86 5y ago
      The above comment is idiotic, yes, but so is this response by France. She cut herself while cleaning a blade, something that doesn't seem so wildly uncommon that an entire nation should stop research on an entire class of diseases because of it.
      - nemetroid 5y ago
        Your reasoning seems backwards to me. If she had hurt herself through some unlikely and intricate series of events, a shutdown would have made less sense.
      - riffraff 5y ago
        I believe the issue is that the kind of precautions they're using were based on some assumptions (e.g. that mouse prions do not affect humans) which were flawed and need to be re-evaluated. It's not just "don't cut yourself, duh".
      - nosianu 5y ago
        That was not the case that prompted this reaction, so I don't understand the reasoning behind your comment? The case that prompted this reaction is described in the first paragraph of the linked article. The one you refer to in the second one as an older case. There is no mention of the older case having lead to any shutdowns, making it hard to follow your train of thoughts, since you specifically refer to this older case.
      - smolder 5y ago
        Maybe researchers who kill themselves with dangerous infectious agents are a sign that precautions are inadequate and deserve scrutiny.
- ashtonkem 5y ago
  At the minimum, some new safety protocols might be needed.
Reventlov 5y ago
From Wikipedia [1]: « Prions are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several atal and transmissible neuro degenerative diseases in humans and many other animals. It is not known what causes the normal protein to misfold, but the abnormal three-dimensional structure is suspected of conferring infectious properties, collapsing nearby protein molecules into the same shape. […] There are no effective treatments for prion diseases. » Basically, you get infected, you die. See also [2]. [1]: https://en.wikipedia.org/wiki/Prion https://en.wikipedia.org/wiki/Prion [2]: https://blogs.scientificamerican.com/artful-amoeba/prions-are-forever/ https://blogs.scientificamerican.com/artful-amoeba/prions-ar...
- bb123 5y ago
  Well we don't really know that, because diagnosis is normally confirmed post-mortem. There could be many people walking around infected with prions that show no symptoms at all.
  - deleted 5y ago
    [deleted]
  - curryst 5y ago
    There's some pedantry here. There is evidence that some people have a mutation that prevents them from getting CJD. The mutation causes a small change in the proteins that CJD prions impact (changing a glycine to valine at a certain part of the protein). The working theory is that that structural change prevents CJD prions from being able to re-form healthy proteins, preventing the spread of the disease. I wouldn't be at all surprised if those proteins are eventually broken down by the body and removed. All that being said, I don't know if you would even consider it an "infection" if the person is immune to the prions spreading. Prions aren't generally transmissible, so if the prions don't do anything and can't be transmitted... is anything actually wrong? You and I are exposed to tons of bacteria every day, but as long as your body can fight it off (i.e. no symptoms and no transmission), we wouldn't usually call that an "infection". In the same light, presence of abnormal proteins that aren't causing an issue doesn't seem like something we would call an "infection". There's also the fact that some people inherit the disease (as in their body actually makes the prions itself), which doesn't make any sense in the context of an "infection". It's their own body producing the prions, so in those cases it's a genetic disease, not an infection.
imglorp 5y ago
> She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds. Does bleach even affect proteins? Isn't that protocol for an organism you're afraid of?
- roflc0ptic 5y ago
  yes, bleach kills microorganisms by denaturing their proteins.
  - Scoundreller 5y ago
    And by oxidizing everything else. It’s not denaturation that’s making my clothes white again.
  - rcxdude 5y ago
    Yes, though prions are extremely tough by protein standards (surviving extremely high temperatures and acidity, making them difficult to clean away. Prions have been transmitted from patient to patient through sterilized instruments used in brain surgery)
- meepmorp 5y ago
  Hypochlorous acid denatures proteins, so yeah.
  - DoingIsLearning 5y ago
    But wasn't there an issue before of prion contamination with surgical instruments used in ophtalmic surgery. I am sure those would have been autoclaved and reprocessed at some ridiculous pH value, no?
    - throwaway0a5e 5y ago
      >I am sure those would have been autoclaved and reprocessed at some ridiculous pH value, no? Probably not until they had reason to do so. "Hot acidic environment" is hell when it comes to corrosion and the engineering incentive for medical stuff is only to be as corrosion resistant as you need because there are other design considerations and the most corrosion resistant stainless steels are not the bet fit for building a super precise needle you're gonna poke around in someone's eye so you wind up wanting to pick a middle ground alloy. Picking a middle ground alloy puts a hard limit on what you can do to clean your instruments before you degrade them in unacceptably short order. I don't know how things have changed since. My medical manufacturing experience is out of date and limited.
AbrahamParangi 5y ago
The study of rare, contagious diseases seems likely enough to result in an eventual outbreak that it should be treated like enriching uranium. Which is to say, with great caution- and not like regular science but rather like the kind of science with potentially very negative externalities.
- gonzo41 5y ago
  Welcome to the exciting world of duel use microbiology research. There's plenty of people are doing stuff that walks the line between learning new things and creating ways to weaponize germs. It's all a question of good governance.
  - choeger 5y ago
    > duel use microbiology research This typo demands an xkcd!
    - marcellus23 5y ago
      no it doesn't.
- Voloskaya 5y ago
  CJD isn't contagious, unless you start eating the brain of your friends.
  - AbrahamParangi 5y ago
    Yes, it doesn’t seem like an immediate risk but I am making a more general point.
- missedthecue 5y ago
  this is a nitpick, but "negative externality" isn't a synonym for "bad things happening". It describes a market transaction which inherently imposes costs on an unrelated third party. For instance, a steel mill producing rolling stock to sell me releases sulfur into the air that dirties your property, imposing a cost on you. Prion research doesn't necessarily impose costs on anyone.
circlefavshape 5y ago
prions sounds very much like ice-nine
- Tsarbomb 5y ago
  That is an excellent way of thinking about them. For anyone unfamiliar, OP is referring to the Kurt Vonnegut novel which has a fictional state of water (ice-nine) that immediately freezes all other water it comes into contact with into more ice-nine. Edit: I committed a sin and didn't mention the title: Cat's Cradle.
  - choeger 5y ago
    There must be some kind of counter-agent, or life as we know it would not exist.
    - reubenswartz 5y ago
      The counter-agent is that it's fictional. ;-) (We hope.)
    - Koshkin 5y ago
      Not necessarily in a satirical science fiction.
    - Cthulhu_ 5y ago
      To prion diseases? Mainly in that they're not that common, more like a weird aberration than something that actively fights and adapts for its own survival like bacteria and viruses. I mean cancer is not contagious, but it still kills millions a year.
      - FreeFull 5y ago
        Strictly speaking, contagious cancer is a thing, but not in humans. See https://en.wikipedia.org/wiki/Canine_transmissible_venereal_tumor https://en.wikipedia.org/wiki/Canine_transmissible_venereal_...
lab-worker 5y ago
Fear mongering. There was never a pandemic or a wide spread prion outbreak caused by a lab leak. They should listen to the science and the scientists, and not politicize vital research that we need to prevent disease outbreaks.
- ta988 5y ago
  Nobody is talking about a pandemic here. The risk is for people working directly on those materials (and people eating them eventually, but that would be a edge case).
jamespwilliams 5y ago
Hm: "long period of uncertainty began on 31 May 2010, when 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. She pierced two layers of latex gloves and drew blood" Later in the article: ""We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi [neuropathologist at the University of Zurich] says." It sounds like the assumption that mouse-adapted sheep prions can't infect humans may be flawed.
- ekianjo 5y ago
  "infect" is not the right word. Prions are not viruses, they do not replicate, they ravage other proteins in their surroundings by misfolding them over and over again until it becomes a fatal pathology.
  - jamespwilliams 5y ago
    The word "prion" is actually a contraction of the words "protein" and "infection". (edit: although I agree it's not what one might conventionally think of as an "infection")
    - ekianjo 5y ago
      I know, but it's a poor choice of word since it's nothing like viruses or bacteria.
      - nkmnz 5y ago
        Viruses are nothing like bacteria, so it doesn’t make any sense to restrict the term of “infection” to those two.
        Asraelite 5y ago
        It is interesting though that we don't consider things like lead and radiation to be "infections". Is the difference between an infection and poisoning replication?
chrbarrol 5y ago
Has there been any research on Prions as a bio-terrorism weapon? It sounds like you could infect thousands/millions and have a close to 100% death-rate without anyone noticing until many years after the contamination started. Imaging contaminating food/drinking water with the Prion which causes Kuru [1] for example. [1] https://en.wikipedia.org/wiki/Kuru_(disease) https://en.wikipedia.org/wiki/Kuru_(disease)
- Omavel 5y ago
  bio-terrorism is not effective.
  - tyingq 5y ago
    What's "effective" mean in this context? The anthrax letters in the US in 2001 certainly dominated news cycles.
    - ekianjo 5y ago
      And killed very few people. That's what we call terrorism (lots of news coverage for very few deaths) - it's not effective in doing anything but news cycles.
      - tyingq 5y ago
        5 envelopes, 5 dead, 17 more infected.
      - emptyfile 5y ago
        >it's not effective in doing anything but news cycles. That's literally the desired effect. What you're thiking of is war.
        ekianjo 5y ago
        I was referring to the original comment above: > It sounds like you could infect thousands/millions Its not the right way to infect millions of people. It does not scale. Therefore not effective.
marcodiego 5y ago
Scary. I think and airborne human transmissible prion disease that kill in a few years is the kind of event that can bring our society back to prehistoric living in just a few decades. Hope it never happens.
- relax88 5y ago
  Is this even possible? By what mechanism could a prion disease become airborne? Viruses have evolved to more easily infect cells in the respiratory tract for this specific purpose, but prions are much less sophisticated. It seems there may be some evidence they can evolve, but enough to hijack cellular machinery in such a way that they could increase the concentration in the respiratory tract enough to transmit? Seems a bit far fetched but I’m no prion expert.
  - hammock 5y ago
    Maybe we should research it
  - Aqueous 5y ago
    This article from 2011 suggests aerosol transmission in mice: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020930/ https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020930/
    - subroutine 5y ago
      To attenuate fears a bit... Aerosol transmission was proven possible if you're sitting inside a vapor inhalation chamber equipped with a nebulizer spraying out liquid droplets containing >2% w/v brain homogenates from terminally scrapie-sick mice. Rest assured if you somehow get infected by PrPSc through airborne transmission, someone was trying really hard to make that happen.
      - BoxOfRain 5y ago
        >a vapor inhalation chamber equipped with a nebulizer spraying out liquid droplets containing >2% w/v brain homogenates from terminally scrapie-sick mice I have never been more grateful to be a backend web developer where the worst risk to my health is a headache from flakey libraries. This sounds like a terrifying job!
ekianjo 5y ago
Next, maybe time do the same thing for gain-of-function research?
fallingknife 5y ago
> Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds. Sounds like a bad lab that wasn't following safety procedures. But even that is in question: > Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”) Why does every lab have to be shut down for 3 months?
- mschuster91 5y ago
  > Why does every lab have to be shut down for 3 months? Auditing everyone and their processes takes a while.
  - fallingknife 5y ago
    Why would you audit processes when the allegation is that someone got infected because she wasn't following the processes?
    - mschuster91 5y ago
      Part of a process audit is to check compliance, e.g. by counting if there are enough metal gloves per employee - including measuring all hand sizes to check if really every employee even has access to a fitting glove -, by checking if supply orders for PPE match expected consumption, by interviewing current and especially former employees and by checking emails for evidence of staff complaints.
    - frob 5y ago
      It's so that they can figure out why the process wasn't being followed and how it can be improved. Nothing is perfect and accidents are a great catalyst for reexamining your procedures. What could change to make it more likely that the process would be followed? Could we put signs all around places with prion research? Could we implement a buddy system whenever we work with prions? Do metal gloves need to be hung in visible place throughout the lab? Do we keep containers of bleach accessible for when someone pricks their finger? Do we have enough of them and are they visible enough?
fsloth 5y ago
Could a cure be to stop proteosynthesis of the prion-protein? https://blogs.scientificamerican.com/artful-amoeba/prions-are-forever/ https://blogs.scientificamerican.com/artful-amoeba/prions-ar... "This protein, in its healthy, properly-folded state, is, if not trivial, relatively unimportant. Its complete loss is certainly not catastrophic." I have no idea how to block proteosynthesis of a single protein in humans, maybe more biologically attuned HN:ners can comment what it would mean (I'm totally clueless of cellular biology).
- jhgb 5y ago
  It seems to me that in an adult, it may already be too late. How would you remove the existing protein?
  - im3w1l 5y ago
    Maybe it would degrade after some time, or be cleaned up by the body. At worst it should stop the disease progression.
  - subroutine 5y ago
    Proteins have a half life of ~24 hours. CJD has an incubation period of roughly 10 years. If de novo PrP synthesis was somehow halted, the likelihood of developing CJD would drop to zero. On the other hand whatever job PrP was doing will also cease getting done, and its downstream pathways will be disrupted. So there are likely consequences. Now if you could totally shut down PrP synthesis for like a week, then turn it back on, that might work. In theory this is possible using a tet-expression system [1], but I've never worked with a tet system that wasn't leaky. The only sure way would be to KO PrP genes from all cells, then CRISPR it back in a week later. Oh, and I'm talking about cells in a culture dish; this strategy would never work in an adult human. [1] https://www.jax.org/news-and-insights/2015/april/introduction-to-tet-expression-systems https://www.jax.org/news-and-insights/2015/april/introductio...
    - jhgb 5y ago
      > Proteins have a half life of ~24 hours. All of them? I admit that I'm not a biologist, so that's new to me, but this would seem to put our bodies under immense stress if we had to replace half of all our proteins every day. If the average human body has 10 kg of proteins, this would mean the necessity of synthesizing 5 kg per day to prevent your death. Or is this specifically about PrP? If it's true that it doesn't last long, then perhaps stopping its synthesis would be a viable option. Does the misfolded PrP have a different half-life?
wil421 5y ago
Kinda a morbid question but could you just sever the finger moments after the prick? Or would the blood pump so fast that the prion(s) is already in your body.
- connorproctor 5y ago
  They mention in the article that normal protocol is to immediately soak the thumb in bleach. Not sure if that’s actually effective though, or just hopeful.
  - throwawayboise 5y ago
    Wow be glad Trump didn't suggest that.
- carlmr 5y ago
  Just don't use the same saw.
- hammock 5y ago
  At the very least a tourniquet could be considered. It only takes one protein to get through though.
- 0xfaded 5y ago
  My housemate is an organic chemist and from a past conversation apparently there is a cautionary paper regarding a grad student who lost a hand due to delayed amputation of a finger after similarly being jabbed with some nasty chemical. Can’t find the paper unfortunately.
- Dah00n 5y ago
  >would the blood pump so fast that the prion(s) is already in your body. Well, I guess the question is "is it in the blood yet or not". As someone who have had lots of stuff injected into his veins multiple times (cancer treatment) I can state for a fact the from injection until pretty much full body exposure can be counted in seconds on one hand. I'm not a doctor but my guess is it would only work if you cut the finger off pretty much instantly (counting from the time it enters the bloodstream). Of course it would take longer from a small cut than injected into the big pipes. I wouldn't trust it to work after a few seconds though.
brohoolio 5y ago
My heart breaks for these families. I can't help but wonder if the labs are taking the necessary precautions in terms of prion disposal. I can't imagine the prions are allowed down the drain....
dekhn 5y ago
Prions are weird. I was at UCSF during the time they went from a fringe theory to an established fact and a nobel prize. The professor who studied them (Prusiner) had to overcome tons of resistance and the experiments were heroic (often waiting months for mice to die). It's really funny that everybody resisted it until somebody mentioned that there was a plausible physical mechanism of refolding catalysis.
- jtbayly 5y ago
  Makes complete sense that there was resistance until a viable mechanism was proposed. Otherwise it just sounds like voodoo.
  - dekhn 5y ago
    No, because the underlying data demonstrated that there was a statistically significant death effect occurring, with a wide range of evidence implicating prions, before there was a mechanism. You shouldn't require a plausible mechanism to trust your experiments (seen this a hundred times, like in RNA enzymes, heart of the ribosome, various dna-is-the-heritable-agent experimetns).
    - XorNot 5y ago
      Yes you should: because all you have otherwise is a death rate that could be caused by any number of things. Biological organisms die all the time for all sorts of reasons: there's a difference between showing a series of factors lead to an death rate, and proposing the specific mechanism by which that happens and it's important. That light behaves like a wave did not summarily prove there was an aether through which it propagated, and not finding it up-ended physics.
      - dekhn 5y ago
        Not only did they know the death rate, they had an infectious agent in a tube they knew was protein. If you identify a reproducible agent you can study in the lab, you don't need any plausible mechanism. Note that aether was the plausible mechanism, and it mislead people for a long time. The experiment that "disproved" aether just showed that the mechanistic proposal was simple, and wrong.
dmix 5y ago
This reminds me of those early 1900s nuclear researchers who got exposed to radiation poisoning which eventually took a long term toll. The multi-year gap makes this one especially difficult to track down to specific events/exposures.
- natmaka 5y ago
  The 'Radium Girls' cases! https://en.wikipedia.org/wiki/Radium_Girls https://en.wikipedia.org/wiki/Radium_Girls "It is safe"...
JoeCoo7 5y ago
Not sure why she wasn't wearing the proper protection or responded to the exposure correctly (bleach rinse apparently) but this goes to show the importance of following lab safety protocols at all times.
- Splendor 5y ago
  > "She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer."
briantakita 5y ago
These mad scientists need to be stopped. How about all research into gain of function, genetic engineering viruses, prions, etc. need to have a moratorium. There is too much risk & not enough transparency on all levels. We don't hear about all of the problems. I wonder what else is wrong and what happened. Whistleblowers need protection, not punishment.
- myWindoonn 5y ago
  Look up how fluorine, the "devil's chemical", was found and isolated: https://en.wikipedia.org/wiki/Fluorine#Isolation https://en.wikipedia.org/wiki/Fluorine#Isolation They blew themselves up, or worse, in pursuit of scientific knowledge. Without their work, semiconductors and electronics wouldn't be possible, and we wouldn't be here on this forum. You don't have to sacrifice yourself, but don't infringe on those brave folks who are advancing our scientific knowledge by risking their lives. (Although if you're a lab-leak conspiracist, then it's probably too late.)
  - alach11 5y ago
    There's a difference between research on explosives (where the scientist risks their own health) and contagious diseases (where the scientist risks everyone's health).
himinlomax 5y ago
I remember hearing people arguing that the Covid lab leak hypothesis was implausible because of stringent safety measures.
- duxup 5y ago
  I really don't want to go too far down the rabbit hole but IIRC some of the concern was due to a lack of stringent safety measures in a lab / similar labs.
- jhgb 5y ago
  The Covid lab leak hypothesis is primarily implausible because of Occam's razor.
  - programmarchy 5y ago
    The lab leak hypothesis is less plausible than the natural leak hypothesis only if you exclude the fact that gain-of-function research on Coronavirus was being done in the Wuhan Lab. When you include that necessary fact, it becomes the most parsimonious. If this researcher becomes ill with CJD, we'll know it's because, beyond a reasonable doubt, she was mishandling prions in a laboratory setting, and not because she ate some infected meat from a bazaar.
    - jhgb 5y ago
      > When you include that necessary fact, it becomes the most parsimonious. That's just wishful thinking on your part. That's still not enough evidence in that direction at all. You have large, dense cities in China and people trading wildlife food. There's millions of them in a cramped space. That's a breeding ground for infection, with millions of opportunities for mutations.
  - himinlomax 5y ago
    That is not an argument. It is extremely plausible because the epidemic started next to the lab. Look at the size of China. Take a map of the country and throw a dart at it. Now consider the probability of it landing within 1 mm of Wuhan. Let's call this dart Occam's fléchette.
detritus 5y ago
This coming so close after the announcement by DeepMind that they're releasing the data on hundreds of thousands of protein folding structures has me with a growing knot in my stomach. As generous and useful on a human-scale an announcement as that was, my first thought was how it could be abused. So too prion research. Who'd've thunk that muscle bulk could be so scary? (!)
- peytoncasper 5y ago
  I just wanted to add a comment about your muscle bulk comment. Protein in protein shakes is usually denatured or rather just a collection of amino acids. Proteins that are synthesized by the body have a structure to them and Prions are simply misfolded representations of those proteins. The process of destroying a Prion involves denaturing it by removing its structure. I don't believe that muscle tissue is anymore related to Prions or structured proteins than any other part of your body as the protein that we eat is simply amino acids or building blocks for creating things like tissues. It does beg the question though, could some of the advancements by DeepMind help us understand how to unfold Prions more effectively than using Bleach.
  - detritus 5y ago
    Just for clarity - my last line there was me being facetious! I was under the impression that even boiling or bleaching did little to kill prions? I seem to recall that even autoclaving surgey equipment did nothing to them, so they had to be thrown? Otherwise, yes - there're lands of opportunity in the science of protein un/folding.. but as much, there's scope for naughtiness too. Seems to me like this level of tech - or, at least - that which may be available in this domain over the next 50 years, could be one of those existential Great Selector/Filter events that biological civilisations might face over their evolution.
    - peytoncasper 5y ago
      Ah apologies. And Bleach is utilized in the labs to denature the prions appropriately. You're correct that autoclaves are not used as heat unfortunately is not enough to kill them. Its like 100% bleach.
tomrod 5y ago
Prions are system glitches. It's nuts to me to think that a complex mix of chemicals can cause slow but fundamental decoherence in a living system.
_user112 5y ago
Timing is weird. Why amidst propaganda and conspiracies pushing experimental mRNA injections?
opwieurposiu 5y ago
Apparently some humans are running a patch that fixes this prion bug. How do we get this merged into mainline? https://www.nature.com/articles/nature.2015.17725 https://www.nature.com/articles/nature.2015.17725
- newsbinator 5y ago
  That's a great question. I would really love an answer. > CRISPR-Cas9-Based Knockout of the Prion Protein and Its Effect on the Proteome https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0114594 https://journals.plos.org/plosone/article?id=10.1371/journal...
  - neolog 5y ago
    That's a nice mechanism but wouldn't we all have to rebase from lucy?
- galactus 5y ago
  We sterilize everyone but them?
RalfWausE 5y ago
My horror vision after clicking the link: Imagine this merges with covid and the whole John Titor fluff becomes instantly more realistic...
- sigstoat 5y ago
  > Imagine this merges with covid imagine the terminator and the alien merge! that's not how anything works.
  - RalfWausE 5y ago
    >imagine the terminator and the alien merge! It DID happen... and it was terrible: https://www.imdb.com/title/tt0112320/mediaviewer/rm35735552/ https://www.imdb.com/title/tt0112320/mediaviewer/rm35735552/
shawnz 5y ago
Somewhat off-topic, but what are the chances that new advancements in protein folding research (like AlphaFold) might let us find cures for prion diseases?
croddin 5y ago
I wonder if it would be possible to make a designer protein that latches onto prion “seed crystals” to neutralize it. Some of the recent advances in protein folding and crispr make it so this might be starting to be something in the realm of possibility.
- droopyEyelids 5y ago
  Gotta be some of the most terrifying technology imaginable.
  - croddin 5y ago
    That idea would be to cure it, prions are terrifying.
redleggedfrog 5y ago
I feel for Émilie. Those must have been tough years.
yarg 5y ago
Time to develop protocols with slice resistant gloves.
zelienople 5y ago
I read Prusiner's book a few years ago and I was disappointed with his inability to definitively satisfy Koch's Postulates. It looks like we can close the chapter on that one, horrifyingly.
stjohnswarts 5y ago
It's only 3 months, sounds like the right thing to do for now.

391 points by ta988 5y ago

The article talks about the case of Émilie Jaumain that got contaminated by prion while cleaning a device used to slice mouse brains. She died 9 years later of a prion disease. After several cases of exposure across the world, France decided to block prion research for 3 months to try to study the issue.

tinus_hn 5y ago
They are going to stop studying so they can start studying the issue? Ah well, this is the level of ‘science’ we’ve come to expect in the pandemic.
- Reventlov 5y ago
  No, they're gonna stop studying until they have reviewed all the security protocols regarding the handling of prions, and this, nationwide.
  - aqsalose 5y ago
    There are reviews and then there are reviews: Rubber stamp security reviews happen. I don't know much about this particular case, but I think, in general, a trustworthy review needs to be conducted by an independent body with genuine power (edit. and desire) to order things to halt permanently if they can not be made safe enough (and then there is a separate question of who makes the call "what is safe enough").
    - bluGill 5y ago
      >(and then there is a separate question of who makes the call "what is safe enough"). This is hard. If there isn't the right incentive they can decide nothing is safe enough. This ensures safety now, but it means that if a cure is possible it won't be discovered (or anything else useful from the research). The FDA has been accused of needlessly holding up useful drugs on this grounds (I'm not going to comment on if true or not)
  - antattack 5y ago
    They want to make sure that disease did not come from other places than the lab, which would be a concern to the general public. However unlikely, it's a possibility that has to be eliminated.
- me_me_me 5y ago
  Yes, they should stop potentially lethal research procedures to see how they can prevent them. Its not rocket science.
  - pc86 5y ago
    The above comment is idiotic, yes, but so is this response by France. She cut herself while cleaning a blade, something that doesn't seem so wildly uncommon that an entire nation should stop research on an entire class of diseases because of it.
    - nemetroid 5y ago
      Your reasoning seems backwards to me. If she had hurt herself through some unlikely and intricate series of events, a shutdown would have made less sense.
    - riffraff 5y ago
      I believe the issue is that the kind of precautions they're using were based on some assumptions (e.g. that mouse prions do not affect humans) which were flawed and need to be re-evaluated. It's not just "don't cut yourself, duh".
    - nosianu 5y ago
      That was not the case that prompted this reaction, so I don't understand the reasoning behind your comment? The case that prompted this reaction is described in the first paragraph of the linked article. The one you refer to in the second one as an older case. There is no mention of the older case having lead to any shutdowns, making it hard to follow your train of thoughts, since you specifically refer to this older case.
    - smolder 5y ago
      Maybe researchers who kill themselves with dangerous infectious agents are a sign that precautions are inadequate and deserve scrutiny.
ashtonkem 5y ago
At the minimum, some new safety protocols might be needed.

Reventlov 5y ago

From Wikipedia [1]: « Prions are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several atal and transmissible neuro degenerative diseases in humans and many other animals. It is not known what causes the normal protein to misfold, but the abnormal three-dimensional structure is suspected of conferring infectious properties, collapsing nearby protein molecules into the same shape. […] There are no effective treatments for prion diseases. » Basically, you get infected, you die. See also [2]. [1]: https://en.wikipedia.org/wiki/Prion https://en.wikipedia.org/wiki/Prion [2]: https://blogs.scientificamerican.com/artful-amoeba/prions-are-forever/ https://blogs.scientificamerican.com/artful-amoeba/prions-ar...

bb123 5y ago
Well we don't really know that, because diagnosis is normally confirmed post-mortem. There could be many people walking around infected with prions that show no symptoms at all.
- deleted 5y ago
  [deleted]
- curryst 5y ago
  There's some pedantry here. There is evidence that some people have a mutation that prevents them from getting CJD. The mutation causes a small change in the proteins that CJD prions impact (changing a glycine to valine at a certain part of the protein). The working theory is that that structural change prevents CJD prions from being able to re-form healthy proteins, preventing the spread of the disease. I wouldn't be at all surprised if those proteins are eventually broken down by the body and removed. All that being said, I don't know if you would even consider it an "infection" if the person is immune to the prions spreading. Prions aren't generally transmissible, so if the prions don't do anything and can't be transmitted... is anything actually wrong? You and I are exposed to tons of bacteria every day, but as long as your body can fight it off (i.e. no symptoms and no transmission), we wouldn't usually call that an "infection". In the same light, presence of abnormal proteins that aren't causing an issue doesn't seem like something we would call an "infection". There's also the fact that some people inherit the disease (as in their body actually makes the prions itself), which doesn't make any sense in the context of an "infection". It's their own body producing the prions, so in those cases it's a genetic disease, not an infection.

imglorp 5y ago

> She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds. Does bleach even affect proteins? Isn't that protocol for an organism you're afraid of?

roflc0ptic 5y ago
yes, bleach kills microorganisms by denaturing their proteins.
- Scoundreller 5y ago
  And by oxidizing everything else. It’s not denaturation that’s making my clothes white again.
- rcxdude 5y ago
  Yes, though prions are extremely tough by protein standards (surviving extremely high temperatures and acidity, making them difficult to clean away. Prions have been transmitted from patient to patient through sterilized instruments used in brain surgery)
meepmorp 5y ago
Hypochlorous acid denatures proteins, so yeah.
- DoingIsLearning 5y ago
  But wasn't there an issue before of prion contamination with surgical instruments used in ophtalmic surgery. I am sure those would have been autoclaved and reprocessed at some ridiculous pH value, no?
  - throwaway0a5e 5y ago
    >I am sure those would have been autoclaved and reprocessed at some ridiculous pH value, no? Probably not until they had reason to do so. "Hot acidic environment" is hell when it comes to corrosion and the engineering incentive for medical stuff is only to be as corrosion resistant as you need because there are other design considerations and the most corrosion resistant stainless steels are not the bet fit for building a super precise needle you're gonna poke around in someone's eye so you wind up wanting to pick a middle ground alloy. Picking a middle ground alloy puts a hard limit on what you can do to clean your instruments before you degrade them in unacceptably short order. I don't know how things have changed since. My medical manufacturing experience is out of date and limited.

AbrahamParangi 5y ago

The study of rare, contagious diseases seems likely enough to result in an eventual outbreak that it should be treated like enriching uranium. Which is to say, with great caution- and not like regular science but rather like the kind of science with potentially very negative externalities.

gonzo41 5y ago
Welcome to the exciting world of duel use microbiology research. There's plenty of people are doing stuff that walks the line between learning new things and creating ways to weaponize germs. It's all a question of good governance.
- choeger 5y ago
  > duel use microbiology research This typo demands an xkcd!
  - marcellus23 5y ago
    no it doesn't.
Voloskaya 5y ago
CJD isn't contagious, unless you start eating the brain of your friends.
- AbrahamParangi 5y ago
  Yes, it doesn’t seem like an immediate risk but I am making a more general point.
missedthecue 5y ago
this is a nitpick, but "negative externality" isn't a synonym for "bad things happening". It describes a market transaction which inherently imposes costs on an unrelated third party. For instance, a steel mill producing rolling stock to sell me releases sulfur into the air that dirties your property, imposing a cost on you. Prion research doesn't necessarily impose costs on anyone.

circlefavshape 5y ago

prions sounds very much like ice-nine

Tsarbomb 5y ago
That is an excellent way of thinking about them. For anyone unfamiliar, OP is referring to the Kurt Vonnegut novel which has a fictional state of water (ice-nine) that immediately freezes all other water it comes into contact with into more ice-nine. Edit: I committed a sin and didn't mention the title: Cat's Cradle.
- choeger 5y ago
  There must be some kind of counter-agent, or life as we know it would not exist.
  - reubenswartz 5y ago
    The counter-agent is that it's fictional. ;-) (We hope.)
  - Koshkin 5y ago
    Not necessarily in a satirical science fiction.
  - Cthulhu_ 5y ago
    To prion diseases? Mainly in that they're not that common, more like a weird aberration than something that actively fights and adapts for its own survival like bacteria and viruses. I mean cancer is not contagious, but it still kills millions a year.
    - FreeFull 5y ago
      Strictly speaking, contagious cancer is a thing, but not in humans. See https://en.wikipedia.org/wiki/Canine_transmissible_venereal_tumor https://en.wikipedia.org/wiki/Canine_transmissible_venereal_...

lab-worker 5y ago

Fear mongering. There was never a pandemic or a wide spread prion outbreak caused by a lab leak. They should listen to the science and the scientists, and not politicize vital research that we need to prevent disease outbreaks.

ta988 5y ago
Nobody is talking about a pandemic here. The risk is for people working directly on those materials (and people eating them eventually, but that would be a edge case).

jamespwilliams 5y ago

Hm: "long period of uncertainty began on 31 May 2010, when 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. She pierced two layers of latex gloves and drew blood" Later in the article: ""We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi [neuropathologist at the University of Zurich] says." It sounds like the assumption that mouse-adapted sheep prions can't infect humans may be flawed.

ekianjo 5y ago
"infect" is not the right word. Prions are not viruses, they do not replicate, they ravage other proteins in their surroundings by misfolding them over and over again until it becomes a fatal pathology.
- jamespwilliams 5y ago
  The word "prion" is actually a contraction of the words "protein" and "infection". (edit: although I agree it's not what one might conventionally think of as an "infection")
  - ekianjo 5y ago
    I know, but it's a poor choice of word since it's nothing like viruses or bacteria.
    - nkmnz 5y ago
      Viruses are nothing like bacteria, so it doesn’t make any sense to restrict the term of “infection” to those two.
      - Asraelite 5y ago
        It is interesting though that we don't consider things like lead and radiation to be "infections". Is the difference between an infection and poisoning replication?

chrbarrol 5y ago

Has there been any research on Prions as a bio-terrorism weapon? It sounds like you could infect thousands/millions and have a close to 100% death-rate without anyone noticing until many years after the contamination started. Imaging contaminating food/drinking water with the Prion which causes Kuru [1] for example. [1] https://en.wikipedia.org/wiki/Kuru_(disease) https://en.wikipedia.org/wiki/Kuru_(disease)

Omavel 5y ago
bio-terrorism is not effective.
- tyingq 5y ago
  What's "effective" mean in this context? The anthrax letters in the US in 2001 certainly dominated news cycles.
  - ekianjo 5y ago
    And killed very few people. That's what we call terrorism (lots of news coverage for very few deaths) - it's not effective in doing anything but news cycles.
    - tyingq 5y ago
      5 envelopes, 5 dead, 17 more infected.
    - emptyfile 5y ago
      >it's not effective in doing anything but news cycles. That's literally the desired effect. What you're thiking of is war.
      - ekianjo 5y ago
        I was referring to the original comment above: > It sounds like you could infect thousands/millions Its not the right way to infect millions of people. It does not scale. Therefore not effective.

marcodiego 5y ago

Scary. I think and airborne human transmissible prion disease that kill in a few years is the kind of event that can bring our society back to prehistoric living in just a few decades. Hope it never happens.

relax88 5y ago
Is this even possible? By what mechanism could a prion disease become airborne? Viruses have evolved to more easily infect cells in the respiratory tract for this specific purpose, but prions are much less sophisticated. It seems there may be some evidence they can evolve, but enough to hijack cellular machinery in such a way that they could increase the concentration in the respiratory tract enough to transmit? Seems a bit far fetched but I’m no prion expert.
- hammock 5y ago
  Maybe we should research it
- Aqueous 5y ago
  This article from 2011 suggests aerosol transmission in mice: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020930/ https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020930/
  - subroutine 5y ago
    To attenuate fears a bit... Aerosol transmission was proven possible if you're sitting inside a vapor inhalation chamber equipped with a nebulizer spraying out liquid droplets containing >2% w/v brain homogenates from terminally scrapie-sick mice. Rest assured if you somehow get infected by PrPSc through airborne transmission, someone was trying really hard to make that happen.
    - BoxOfRain 5y ago
      >a vapor inhalation chamber equipped with a nebulizer spraying out liquid droplets containing >2% w/v brain homogenates from terminally scrapie-sick mice I have never been more grateful to be a backend web developer where the worst risk to my health is a headache from flakey libraries. This sounds like a terrifying job!

ekianjo 5y ago

Next, maybe time do the same thing for gain-of-function research?

fallingknife 5y ago

> Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds. Sounds like a bad lab that wasn't following safety procedures. But even that is in question: > Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”) Why does every lab have to be shut down for 3 months?

mschuster91 5y ago
> Why does every lab have to be shut down for 3 months? Auditing everyone and their processes takes a while.
- fallingknife 5y ago
  Why would you audit processes when the allegation is that someone got infected because she wasn't following the processes?
  - mschuster91 5y ago
    Part of a process audit is to check compliance, e.g. by counting if there are enough metal gloves per employee - including measuring all hand sizes to check if really every employee even has access to a fitting glove -, by checking if supply orders for PPE match expected consumption, by interviewing current and especially former employees and by checking emails for evidence of staff complaints.
  - frob 5y ago
    It's so that they can figure out why the process wasn't being followed and how it can be improved. Nothing is perfect and accidents are a great catalyst for reexamining your procedures. What could change to make it more likely that the process would be followed? Could we put signs all around places with prion research? Could we implement a buddy system whenever we work with prions? Do metal gloves need to be hung in visible place throughout the lab? Do we keep containers of bleach accessible for when someone pricks their finger? Do we have enough of them and are they visible enough?

fsloth 5y ago

Could a cure be to stop proteosynthesis of the prion-protein? https://blogs.scientificamerican.com/artful-amoeba/prions-are-forever/ https://blogs.scientificamerican.com/artful-amoeba/prions-ar... "This protein, in its healthy, properly-folded state, is, if not trivial, relatively unimportant. Its complete loss is certainly not catastrophic." I have no idea how to block proteosynthesis of a single protein in humans, maybe more biologically attuned HN:ners can comment what it would mean (I'm totally clueless of cellular biology).

jhgb 5y ago
It seems to me that in an adult, it may already be too late. How would you remove the existing protein?
- im3w1l 5y ago
  Maybe it would degrade after some time, or be cleaned up by the body. At worst it should stop the disease progression.
- subroutine 5y ago
  Proteins have a half life of ~24 hours. CJD has an incubation period of roughly 10 years. If de novo PrP synthesis was somehow halted, the likelihood of developing CJD would drop to zero. On the other hand whatever job PrP was doing will also cease getting done, and its downstream pathways will be disrupted. So there are likely consequences. Now if you could totally shut down PrP synthesis for like a week, then turn it back on, that might work. In theory this is possible using a tet-expression system [1], but I've never worked with a tet system that wasn't leaky. The only sure way would be to KO PrP genes from all cells, then CRISPR it back in a week later. Oh, and I'm talking about cells in a culture dish; this strategy would never work in an adult human. [1] https://www.jax.org/news-and-insights/2015/april/introduction-to-tet-expression-systems https://www.jax.org/news-and-insights/2015/april/introductio...
  - jhgb 5y ago
    > Proteins have a half life of ~24 hours. All of them? I admit that I'm not a biologist, so that's new to me, but this would seem to put our bodies under immense stress if we had to replace half of all our proteins every day. If the average human body has 10 kg of proteins, this would mean the necessity of synthesizing 5 kg per day to prevent your death. Or is this specifically about PrP? If it's true that it doesn't last long, then perhaps stopping its synthesis would be a viable option. Does the misfolded PrP have a different half-life?

wil421 5y ago

Kinda a morbid question but could you just sever the finger moments after the prick? Or would the blood pump so fast that the prion(s) is already in your body.

connorproctor 5y ago
They mention in the article that normal protocol is to immediately soak the thumb in bleach. Not sure if that’s actually effective though, or just hopeful.
- throwawayboise 5y ago
  Wow be glad Trump didn't suggest that.
carlmr 5y ago
Just don't use the same saw.
hammock 5y ago
At the very least a tourniquet could be considered. It only takes one protein to get through though.
0xfaded 5y ago
My housemate is an organic chemist and from a past conversation apparently there is a cautionary paper regarding a grad student who lost a hand due to delayed amputation of a finger after similarly being jabbed with some nasty chemical. Can’t find the paper unfortunately.
Dah00n 5y ago
>would the blood pump so fast that the prion(s) is already in your body. Well, I guess the question is "is it in the blood yet or not". As someone who have had lots of stuff injected into his veins multiple times (cancer treatment) I can state for a fact the from injection until pretty much full body exposure can be counted in seconds on one hand. I'm not a doctor but my guess is it would only work if you cut the finger off pretty much instantly (counting from the time it enters the bloodstream). Of course it would take longer from a small cut than injected into the big pipes. I wouldn't trust it to work after a few seconds though.

brohoolio 5y ago

My heart breaks for these families. I can't help but wonder if the labs are taking the necessary precautions in terms of prion disposal. I can't imagine the prions are allowed down the drain....

dekhn 5y ago

Prions are weird. I was at UCSF during the time they went from a fringe theory to an established fact and a nobel prize. The professor who studied them (Prusiner) had to overcome tons of resistance and the experiments were heroic (often waiting months for mice to die). It's really funny that everybody resisted it until somebody mentioned that there was a plausible physical mechanism of refolding catalysis.

jtbayly 5y ago
Makes complete sense that there was resistance until a viable mechanism was proposed. Otherwise it just sounds like voodoo.
- dekhn 5y ago
  No, because the underlying data demonstrated that there was a statistically significant death effect occurring, with a wide range of evidence implicating prions, before there was a mechanism. You shouldn't require a plausible mechanism to trust your experiments (seen this a hundred times, like in RNA enzymes, heart of the ribosome, various dna-is-the-heritable-agent experimetns).
  - XorNot 5y ago
    Yes you should: because all you have otherwise is a death rate that could be caused by any number of things. Biological organisms die all the time for all sorts of reasons: there's a difference between showing a series of factors lead to an death rate, and proposing the specific mechanism by which that happens and it's important. That light behaves like a wave did not summarily prove there was an aether through which it propagated, and not finding it up-ended physics.
    - dekhn 5y ago
      Not only did they know the death rate, they had an infectious agent in a tube they knew was protein. If you identify a reproducible agent you can study in the lab, you don't need any plausible mechanism. Note that aether was the plausible mechanism, and it mislead people for a long time. The experiment that "disproved" aether just showed that the mechanistic proposal was simple, and wrong.

dmix 5y ago

This reminds me of those early 1900s nuclear researchers who got exposed to radiation poisoning which eventually took a long term toll. The multi-year gap makes this one especially difficult to track down to specific events/exposures.

natmaka 5y ago
The 'Radium Girls' cases! https://en.wikipedia.org/wiki/Radium_Girls https://en.wikipedia.org/wiki/Radium_Girls "It is safe"...

JoeCoo7 5y ago

Not sure why she wasn't wearing the proper protection or responded to the exposure correctly (bleach rinse apparently) but this goes to show the importance of following lab safety protocols at all times.

Splendor 5y ago
> "She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer."

briantakita 5y ago

These mad scientists need to be stopped. How about all research into gain of function, genetic engineering viruses, prions, etc. need to have a moratorium. There is too much risk & not enough transparency on all levels. We don't hear about all of the problems. I wonder what else is wrong and what happened. Whistleblowers need protection, not punishment.

myWindoonn 5y ago
Look up how fluorine, the "devil's chemical", was found and isolated: https://en.wikipedia.org/wiki/Fluorine#Isolation https://en.wikipedia.org/wiki/Fluorine#Isolation They blew themselves up, or worse, in pursuit of scientific knowledge. Without their work, semiconductors and electronics wouldn't be possible, and we wouldn't be here on this forum. You don't have to sacrifice yourself, but don't infringe on those brave folks who are advancing our scientific knowledge by risking their lives. (Although if you're a lab-leak conspiracist, then it's probably too late.)
- alach11 5y ago
  There's a difference between research on explosives (where the scientist risks their own health) and contagious diseases (where the scientist risks everyone's health).

himinlomax 5y ago

I remember hearing people arguing that the Covid lab leak hypothesis was implausible because of stringent safety measures.

duxup 5y ago
I really don't want to go too far down the rabbit hole but IIRC some of the concern was due to a lack of stringent safety measures in a lab / similar labs.
jhgb 5y ago
The Covid lab leak hypothesis is primarily implausible because of Occam's razor.
- programmarchy 5y ago
  The lab leak hypothesis is less plausible than the natural leak hypothesis only if you exclude the fact that gain-of-function research on Coronavirus was being done in the Wuhan Lab. When you include that necessary fact, it becomes the most parsimonious. If this researcher becomes ill with CJD, we'll know it's because, beyond a reasonable doubt, she was mishandling prions in a laboratory setting, and not because she ate some infected meat from a bazaar.
  - jhgb 5y ago
    > When you include that necessary fact, it becomes the most parsimonious. That's just wishful thinking on your part. That's still not enough evidence in that direction at all. You have large, dense cities in China and people trading wildlife food. There's millions of them in a cramped space. That's a breeding ground for infection, with millions of opportunities for mutations.
- himinlomax 5y ago
  That is not an argument. It is extremely plausible because the epidemic started next to the lab. Look at the size of China. Take a map of the country and throw a dart at it. Now consider the probability of it landing within 1 mm of Wuhan. Let's call this dart Occam's fléchette.

detritus 5y ago

This coming so close after the announcement by DeepMind that they're releasing the data on hundreds of thousands of protein folding structures has me with a growing knot in my stomach. As generous and useful on a human-scale an announcement as that was, my first thought was how it could be abused. So too prion research. Who'd've thunk that muscle bulk could be so scary? (!)

peytoncasper 5y ago
I just wanted to add a comment about your muscle bulk comment. Protein in protein shakes is usually denatured or rather just a collection of amino acids. Proteins that are synthesized by the body have a structure to them and Prions are simply misfolded representations of those proteins. The process of destroying a Prion involves denaturing it by removing its structure. I don't believe that muscle tissue is anymore related to Prions or structured proteins than any other part of your body as the protein that we eat is simply amino acids or building blocks for creating things like tissues. It does beg the question though, could some of the advancements by DeepMind help us understand how to unfold Prions more effectively than using Bleach.
- detritus 5y ago
  Just for clarity - my last line there was me being facetious! I was under the impression that even boiling or bleaching did little to kill prions? I seem to recall that even autoclaving surgey equipment did nothing to them, so they had to be thrown? Otherwise, yes - there're lands of opportunity in the science of protein un/folding.. but as much, there's scope for naughtiness too. Seems to me like this level of tech - or, at least - that which may be available in this domain over the next 50 years, could be one of those existential Great Selector/Filter events that biological civilisations might face over their evolution.
  - peytoncasper 5y ago
    Ah apologies. And Bleach is utilized in the labs to denature the prions appropriately. You're correct that autoclaves are not used as heat unfortunately is not enough to kill them. Its like 100% bleach.

tomrod 5y ago

Prions are system glitches. It's nuts to me to think that a complex mix of chemicals can cause slow but fundamental decoherence in a living system.

_user112 5y ago

Timing is weird. Why amidst propaganda and conspiracies pushing experimental mRNA injections?

opwieurposiu 5y ago

Apparently some humans are running a patch that fixes this prion bug. How do we get this merged into mainline? https://www.nature.com/articles/nature.2015.17725 https://www.nature.com/articles/nature.2015.17725

newsbinator 5y ago
That's a great question. I would really love an answer. > CRISPR-Cas9-Based Knockout of the Prion Protein and Its Effect on the Proteome https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0114594 https://journals.plos.org/plosone/article?id=10.1371/journal...
- neolog 5y ago
  That's a nice mechanism but wouldn't we all have to rebase from lucy?
galactus 5y ago
We sterilize everyone but them?

RalfWausE 5y ago

My horror vision after clicking the link: Imagine this merges with covid and the whole John Titor fluff becomes instantly more realistic...

sigstoat 5y ago
> Imagine this merges with covid imagine the terminator and the alien merge! that's not how anything works.
- RalfWausE 5y ago
  >imagine the terminator and the alien merge! It DID happen... and it was terrible: https://www.imdb.com/title/tt0112320/mediaviewer/rm35735552/ https://www.imdb.com/title/tt0112320/mediaviewer/rm35735552/

shawnz 5y ago

Somewhat off-topic, but what are the chances that new advancements in protein folding research (like AlphaFold) might let us find cures for prion diseases?

croddin 5y ago

I wonder if it would be possible to make a designer protein that latches onto prion “seed crystals” to neutralize it. Some of the recent advances in protein folding and crispr make it so this might be starting to be something in the realm of possibility.

droopyEyelids 5y ago
Gotta be some of the most terrifying technology imaginable.
- croddin 5y ago
  That idea would be to cure it, prions are terrifying.

redleggedfrog 5y ago

I feel for Émilie. Those must have been tough years.

yarg 5y ago

Time to develop protocols with slice resistant gloves.

zelienople 5y ago

I read Prusiner's book a few years ago and I was disappointed with his inability to definitively satisfy Koch's Postulates. It looks like we can close the chapter on that one, horrifyingly.

stjohnswarts 5y ago

It's only 3 months, sounds like the right thing to do for now.