[HN Gopher] France issues moratorium on prion research after fat...
       ___________________________________________________________________
        
       France issues moratorium on prion research after fatal brain
       disease strikes
        
       Author : ta988
       Score  : 317 points
       Date   : 2021-07-28 12:34 UTC (6 hours ago)
        
 (HTM) web link (www.sciencemag.org)
 (TXT) w3m dump (www.sciencemag.org)
        
       | croddin wrote:
       | 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 wrote:
         | Gotta be some of the most terrifying technology imaginable.
        
           | croddin wrote:
           | That idea would be to cure it, prions are terrifying.
        
       | AbrahamParangi wrote:
       | 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 wrote:
         | 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 wrote:
           | > duel use microbiology research
           | 
           | This typo demands an xkcd!
        
             | marcellus23 wrote:
             | no it doesn't.
        
         | Voloskaya wrote:
         | CJD isn't contagious, unless you start eating the brain of your
         | friends.
        
           | AbrahamParangi wrote:
           | Yes, it doesn't seem like an immediate risk but I am making a
           | more general point.
        
         | missedthecue wrote:
         | 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.
        
           | zzbzq wrote:
           | It's not a nitpick, it's just wrong. Bad things happening is
           | a cost which is both negative and external. The use of the
           | term "negative externality" works here.
        
             | missedthecue wrote:
             | Negative externality is an economic term which describes a
             | specific market failure. A research lab leak (or nuclear
             | meltdown, or whatever) is a rare accident, not a negative
             | externality.
             | 
             | It would be like me saying the theory of relativity just
             | means a theory about how everything is relative. That's
             | obviously wrong. You can't just break down academic terms
             | into their separate words. It doesn't make sense.
        
               | jjk166 wrote:
               | Third parties suffering a disease is the negative
               | externality.
               | 
               | There's no difference between a steel mill leaking
               | sulphur in the course of producing steel and a biotech
               | company releasing prions in the course of producing
               | medicines. Radiation leaks in the course of producing
               | electricity are likewise externalities.
        
               | missedthecue wrote:
               | Yes, there is a main difference which changes the entire
               | definition. Sulfur emissions are a core part of steel
               | production. You cannot manufacture steel without using
               | carbon in the reduction process. It is inherent.
               | 
               | However, you CAN research diseases without having
               | accidental lab leaks. Thousands of labs around the world
               | do this perfectly fine year in, year out. That's the way
               | it's normally done. The process of researching diseases
               | does not inherently impose costs on anyone else.
        
               | jjk166 wrote:
               | No, sulfur release is not a core part of steel
               | production, and indeed sulphur emmision has been
               | drastically reduced since regulations made steel
               | producers bear the cost of sulphur emmissions, removing
               | it as an externality.
               | 
               | Labs don't continuously spew out diseases, but they very
               | much must impose the risk of such releases upon others.
               | That is the cost. It costs money to insure against risk,
               | even if the disaster never comes to pass. If a biotech
               | company reduces its costs by implementing looser safety
               | policies thus increasing risk to the surrounding
               | population, that cost is borne externally. Hence the
               | name.
        
               | AbrahamParangi wrote:
               | What I am arguing is that low-probability events can be
               | treated as effectively certain over large enough sample
               | sizes.
               | 
               | Similarly, dangerous jobs impose a societal health burden
               | via workplace accidents. Each accident is one-off but in
               | aggregate, a non-zero accident rate is a certain outcome.
               | 
               | Containment breaches of contagious diseases, while each a
               | one-off event, are effectively certain over large enough
               | sample sizes- and therefore should be considered as part
               | of the cost of doing research into them.
        
         | harles wrote:
         | Known prions can't cause an outbreak without extraordinary
         | methods of spread, such as cannibalism.
        
           | XorNot wrote:
           | Also no terrorist is interested in a weapon which takes 10
           | years to kill their target population, maybe.
           | 
           | The threat of WMD terror hasn't materialized in a meaningful
           | way: intact, state-manufactured nuclear weapons are the only
           | plausible weapons non-state actors can realistically handle
           | without killing themselves with them, and even that's pretty
           | questionable: it's not _easy_ to detonate a nuclear bomb.
           | 
           | Chemicals are even worse: it's pretty easy for a chemistry
           | student to accidentally make organophosphates, but terror
           | groups don't try in any substantial way - you're as likely to
           | poison yourself as anyone else.
           | 
           | Biologicals are the worst: screw up and they infect _you_ and
           | then the rest of your organisation - or you have a power cut
           | and everything dies in the test tube.
        
             | meowface wrote:
             | >Also no terrorist is interested in a weapon which takes 10
             | years to kill their target population, maybe.
             | 
             | Not necessarily. If you can somehow pull off a food supply
             | chain attack which creates the perception that a lot of
             | food may have been contaminated with prions, that could
             | potentially very effectively induce a state of mass terror.
             | Symptoms take years to manifest; can't be fully diagnosed
             | until you're already dead; inevitably fatal.
             | 
             | It could potentially turn half of a country into (somewhat
             | rational) hypochondriacs, even if almost no one actually
             | became infected. And if it were confirmed that a lot of
             | people were infected, that'd ramp up the fear much more.
             | Also, the cost of pulling it off is probably much lower
             | than the cost of detonating a dirty bomb or even a full
             | nuclear bomb, while potentially achieving a much stronger
             | effect.
             | 
             | >The threat of WMD terror hasn't materialized in a
             | meaningful way: intact, state-manufactured nuclear weapons
             | are the only plausible weapons non-state actors can
             | realistically handle without killing themselves with them,
             | and even that's pretty questionable: it's not easy to
             | detonate a nuclear bomb.
             | 
             | I kind of disagree. Nuclear weapons seem like they probably
             | won't be viable for terrorism for a very long time, if
             | ever, but I don't think they're the only plausible weapons
             | that can realistically be handled. Look at Aum Shinrikyo
             | and their various planned, attempted, and successful
             | chemical and biological attacks in the 90s. They killed 27
             | and injured over 6,000 people, but they came very close to
             | potentially killing or debilitating tens of thousands.
             | 
             | There's definitely a risk of being injured or dying in the
             | process, but they took (largely effective) precautions to
             | prevent that. You can also do things like use binary agents
             | which only activate when combined. But, perhaps most
             | importantly, terrorists (regardless of motive/ideology)
             | often fully accept and welcome that they may be harmed or
             | killed in the process, so the risk isn't a big deterrent.
             | 
             | I suspect that within this century, at least one
             | terrorist/cult/lone wolf chemical and/or biological attack
             | is going to come close to, or succeed at, killing a large
             | number of people, and I think the threat of future attacks
             | may paralyze a lot of societies.
        
           | neolog wrote:
           | CJD is heritable.
        
       | shawnz wrote:
       | 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?
        
       | marcodiego wrote:
       | 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.
        
         | triceratops wrote:
         | I find prions unbelievably scary too. What reassures me is that
         | they've had about 3 billion years to wipe out life on earth and
         | haven't done it yet. Which means there must be some natural
         | limits on their spread we don't know about yet.
        
           | da_big_ghey wrote:
           | Because we have had many population isolated. Indians never
           | got black plague because Europeans isolated, but got smallpox
           | when Europeans show up. Syphilis was deadly until making of
           | penicilin, probably came back with Europeans from Indians. If
           | whole world is connected then disease maybe can go through
           | whole population.
        
             | inglor_cz wrote:
             | Prions infect animals of all sorts as well, so isolation of
             | human populations is of lesser importance here.
             | 
             | If, say, oceanic fish were susceptible to some easily
             | transmitted prions, you would have a worldwide epidemics in
             | no time. Fish form a crucial part of almost all food chains
             | on the planet.
        
             | 8note wrote:
             | I thought syphilis was from America, not India?
             | 
             | The Indians still had trade going with the middle east
             | during the black plague, but by some magic it doesn't seem
             | to have made it by land or sea
        
               | karlkeefer wrote:
               | I think they mean American Indians aka native Americans.
        
         | newacct583 wrote:
         | Prions aren't airborne. vCJD and mad cow spread by a feedback
         | loop involving slaughtered infected animal brains being fed to
         | the same animal stock. This case was a technician who stabbed
         | herself with a tool heavily coated in brain matter from
         | infected animals. It's not like a virus where just a few
         | particles is enough to bootstrap an infection. At least, it's
         | never been seen.
        
         | relax88 wrote:
         | 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 wrote:
           | Maybe we should research it
        
           | Aqueous wrote:
           | This article from 2011 suggests aerosol transmission in mice:
           | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020930/
        
             | subroutine wrote:
             | 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 wrote:
               | >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!
        
             | learc83 wrote:
             | They used a nebulizer to spray infected brain matter into
             | the air. There was no aerosolized mouse to mouse transfer.
        
       | circlefavshape wrote:
       | prions sounds very much like ice-nine
        
         | gilbetron wrote:
         | You should read about strange matter/strangelets:
         | 
         | https://en.wikipedia.org/wiki/Strangelet#Dangers
        
         | Tsarbomb wrote:
         | 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 wrote:
           | There must be some kind of counter-agent, or life as we know
           | it would not exist.
        
             | reubenswartz wrote:
             | The counter-agent is that it's fictional. ;-) (We hope.)
        
             | Koshkin wrote:
             | Not necessarily in a satirical science fiction.
        
             | Cthulhu_ wrote:
             | 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 wrote:
               | Strictly speaking, contagious cancer is a thing, but not
               | in humans. See https://en.wikipedia.org/wiki/Canine_trans
               | missible_venereal_...
        
               | xvilka wrote:
               | Yet.
        
       | ekianjo wrote:
       | Next, maybe time do the same thing for gain-of-function research?
        
       | lab-worker wrote:
       | 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 wrote:
         | 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).
        
       | RalfWausE wrote:
       | My horror vision after clicking the link: Imagine this merges
       | with covid and the whole John Titor fluff becomes instantly more
       | realistic...
        
         | sigstoat wrote:
         | > Imagine this merges with covid
         | 
         | imagine the terminator and the alien merge!
         | 
         | that's not how anything works.
        
           | RalfWausE wrote:
           | >imagine the terminator and the alien merge!
           | 
           | It DID happen... and it was terrible:
           | 
           | https://www.imdb.com/title/tt0112320/mediaviewer/rm35735552/
        
       | _user112 wrote:
       | Timing is weird.
       | 
       | Why amidst propaganda and conspiracies pushing experimental mRNA
       | injections?
        
       | fallingknife wrote:
       | > 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?
        
         | mannykannot wrote:
         | Your second quote is the reason why: the administrative
         | controls that are supposed to prevent this sort of incident at
         | any of the labs inarguably failed in this case, and there is no
         | reason to believe they will not fail again.
         | 
         | In addition, these controls may be based on false and overly-
         | optimistic assumptions about the risk these pathogens pose.
         | 
         | For either of these reasons, returning to the status quo ante
         | may not be an adequate response. In general, administrative and
         | procedural controls are inferior to uncircumventable physical
         | barriers.
        
         | mschuster91 wrote:
         | > Why does every lab have to be shut down for 3 months?
         | 
         | Auditing everyone and their processes takes a while.
        
           | fallingknife wrote:
           | Why would you audit processes when the allegation is that
           | someone got infected because she wasn't following the
           | processes?
        
             | marcellus23 wrote:
             | She hadn't been trained in how to handle prions. Doesn't
             | that sound like a process problem to you?
        
             | mschuster91 wrote:
             | 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.
        
             | jjk166 wrote:
             | Someone not following a process is a failure of the
             | process. The process should include proper training and
             | regular oversight to ensure that the process is being
             | followed - an audit will reveal if that training and
             | oversight is in place. If you trace any accident back far
             | enough, you'll eventually get to a point where it could've
             | been prevented with better planning.
        
             | frob wrote:
             | 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?
        
               | fallingknife wrote:
               | The last thing you need is more bureaucracy. If you want
               | the rules to be followed, you punish the people who
               | don't. That's it. It doesn't take 3 months. It doesn't
               | take any audits or committees. If there is a rule
               | following problem, you enforce the rules. People will
               | find a way to comply if they know they will pay the price
               | if they don't.
        
             | da_big_ghey wrote:
             | if we have some dangerous prion, and some person is
             | bypassing procedure, that still is needing some evaluation.
             | prion escape and becoming common in populations would be a
             | large disaster.
        
               | mschuster91 wrote:
               | > prion escape and becoming common in populations would
               | be a large disaster.
               | 
               | IIRC, all known prion diseases are only transmissible by
               | ingestion of infectious particles - meaning, eating from
               | infected parts of a carcass, urine/feces and blood.
        
               | mannykannot wrote:
               | There does not seem to have been any ingestion in these
               | two cases. Still, they do not involve transmission
               | through air or through casual contact, but see the last
               | couple of sentences here with regard to the former:
               | https://news.ycombinator.com/item?id=27984942
        
         | josefx wrote:
         | I think the report finding that there were no safety violations
         | is the most damning. You have 100 researchers dealing with a
         | deadly disease, 17 had lab accidents during their work, 5 of
         | them may have suffered fatal exposure with no training how to
         | mitigate this? What are the mandated safety procedures if none
         | where violated? Wear a labcoat and pray that you wont die ten
         | years from now?
        
         | perlgeek wrote:
         | Risk management can (and often does) include simply accepting
         | risks and their consequences.
         | 
         | A strong risk management doesn't necessarily mean strong
         | protection of employees, just that lots of risks have been
         | considered, documented and weighed in some form.
        
       | himinlomax wrote:
       | I remember hearing people arguing that the Covid lab leak
       | hypothesis was implausible because of stringent safety measures.
        
         | jhgb wrote:
         | The Covid lab leak hypothesis is _primarily_ implausible
         | because of Occam 's razor.
        
           | programmarchy wrote:
           | 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.
        
           | himinlomax wrote:
           | 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 flechette.
        
         | duxup wrote:
         | 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.
        
       | ta988 wrote:
       | The article talks about the case of Emilie 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.
        
         | ashtonkem wrote:
         | At the minimum, some new safety protocols might be needed.
        
         | tinus_hn wrote:
         | 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.
        
           | me_me_me wrote:
           | Yes, they should stop potentially lethal research procedures
           | to see how they can prevent them.
           | 
           | Its not rocket science.
        
             | pc86 wrote:
             | 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 wrote:
               | 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 wrote:
               | 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 wrote:
               | 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 wrote:
               | Maybe researchers who kill themselves with dangerous
               | infectious agents are a sign that precautions are
               | inadequate and deserve scrutiny.
        
           | Reventlov wrote:
           | No, they're gonna stop studying until they have reviewed all
           | the security protocols regarding the handling of prions, and
           | this, nationwide.
        
             | aqsalose wrote:
             | 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 wrote:
               | >(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 wrote:
             | 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.
        
       | detritus wrote:
       | 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 wrote:
         | 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 wrote:
           | 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 wrote:
             | 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 wrote:
       | 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.
        
       | jamespwilliams wrote:
       | 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.
        
         | jonplackett wrote:
         | "Never been shown to" is the kind of false speak I keep hearing
         | from the government and media.
         | 
         | Just because you've never tried it doesn't mean you have shown
         | something.
        
           | jarvic wrote:
           | There is a difference between "never been shown to" and
           | "shown to never".
        
             | acoard wrote:
             | Scientists (and logicians) avoid saying "shown to never",
             | as it's much harder - almost impossible - to prove a
             | negative[0]. This is also a black swan[1] type situation.
             | The only way you can say something is never something is if
             | you understand all occurrences of it; otherwise you can
             | only report on what you've been shown.
             | 
             | [0] https://en.wikipedia.org/wiki/Burden_of_proof_(philosop
             | hy)#P...
             | 
             | [1] https://en.wikipedia.org/wiki/Black_swan_theory
        
               | coldtea wrote:
               | > _This is also a black swan[1] type situation_
               | 
               | So one that is almost certain to happen given some time?
               | 
               | > _The only way you can say something is never something
               | is if you understand all occurrences of it;_
               | 
               | "Understanding all occurences of it" seems like a good
               | criterion for allowing potentially mass murderous work or
               | not. That said, it sounds stricter than what it is. We
               | just need them to know how a thing works and not be doing
               | "sorcerer's apprentices" style peek and poke work.
               | 
               | They don't need to "prove a negative". For example we
               | don't asks scientists to prove that "water can't explode
               | and destroy the earth" to let them work with it casually.
               | It's enough that they know it well. Do they know prion as
               | well as they do water?
        
               | BoiledCabbage wrote:
               | Yes but the problem with "never been shown" is how it's
               | often used to "take advantage" of people's interpretation
               | like what you list above.
               | 
               | For example dropping a 27.4 kg ball on someone's head has
               | never been shown to cause injury. We've dropped a 26.3
               | and a 29.5, but we've never studied dropping 27.4 kg -
               | and as a result, "dropping a 27.4 kg ball on someone's
               | head has never been shown to cause injury".
               | 
               | Frequently "never been shown" its used as a weasely way
               | of saying "we've never looked at", or "we don't know if"
        
             | jonplackett wrote:
             | That was meant to be my point, the down vote makes me think
             | I didn't make it clearly enough...
        
             | pmichaud wrote:
             | You're absolutely right, but it's also true that the phrase
             | "never been shown to" most often functions in a sentence
             | more like "doesn't/can't happen" than "we have no idea."
        
             | Symmetry wrote:
             | Yes but many experts take "never been shown to" to mean
             | that we ought to act as if it doesn't for now. That's an
             | important and useful social mechanism in the laborious
             | process of constructing a solid edifice of truth from the
             | crooked timber of humanity. But it's unwise when
             | considering public health responses.
        
           | bitL wrote:
           | "There is no evidence that..." is my recent favorite.
        
             | jonplackett wrote:
             | Yeah that's been constant. There was 'no evidence that
             | delta variant is more dangerous' for white a while, until
             | there was actually some evidence to go on.
        
         | philshem wrote:
         | The full section with the Aguzzi quote adds context, including
         | his declining to comment on the French case.
         | 
         | > The scientific community has long recognized that handling
         | prions is dangerous and an occupational risk for
         | neuropathologists, says neuropathologist Adriano Aguzzi of the
         | University of Zurich. 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. In a
         | 2011 paper, his team reported that prions can spread through
         | aerosols, at least in mice, which "may warrant re-thinking on
         | prion biosafety guidelines in research and diagnostic
         | laboratories," they wrote. Aguzzi says he was "totally shocked"
         | by the finding and introduced safety measures to prevent
         | aerosol spread at his own lab, but the paper drew little
         | attention elsewhere.
        
         | echelon wrote:
         | When lab workers studying the very thing they know best can
         | make lethal mistakes, it's surprising that so many jumped to
         | quell any hypothesis of a covid lab leak.
         | 
         | Scientists make mistakes all the time. They're human.
         | 
         | Imagine all of the mistakes you make in your job. Outages.
         | Stupid bugs. It's the same with any profession.
        
           | knownjorbist wrote:
           | You are conflating lab leak with intentionally crafted
           | bioweapon.
        
             | l30n4da5 wrote:
             | > You are conflating lab leak with intentionally crafted
             | bioweapon.
             | 
             | > intentionally crafted bioweapon
             | 
             | they didn't mention anything about that anywhere.
        
             | echelon wrote:
             | A bioweapon that can't be targeted doesn't seem very
             | useful, does it?
        
               | yonaguska wrote:
               | Could easily be both. Bioweapon development that was
               | accidentally leaked.
        
               | [deleted]
        
             | garmaine wrote:
             | Is he? He only talked about lab leaks. You brought up the
             | nonsense bioweapon theory.
        
               | x86_64Ubuntu wrote:
               | But then he goes to the HN endorsed point of "lab leak"
               | hypothesis.
        
         | ekianjo wrote:
         | "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.
        
           | dekhn wrote:
           | Techynically you are incorrect. Infect is the right word.
           | Worked at the institution that established this. (I do not
           | believe that koch's postulates cover all cases of
           | 'infection').
        
           | LatteLazy wrote:
           | Don't prions reproduce by re-folding other proteins to match
           | themselves?
        
             | gostsamo wrote:
             | Infection means contamination / spoilage.
             | 
             | https://www.etymonline.com/word/infection
        
               | jjk166 wrote:
               | Prions contaminate
        
           | ekster wrote:
           | Infect is definitely the right word here.
        
           | robbrown451 wrote:
           | Prions are considered infectious, although when they were
           | first proposed it was essentially heresy that something could
           | be infectious without containing any nucleic acid (DNA/RNA).
           | 
           | Whether they self-replicate or not is sort of a semantic
           | issue. They replicate in the same way "fallen-down dominos"
           | replicate.
           | 
           | Prior to being a prion, they are an already complex protein,
           | but by folding a particular way, they become a prion, which
           | causes other proteins of the same type to fold similarly.
        
           | jamespwilliams wrote:
           | 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")
        
             | fabiospampinato wrote:
             | What things are named and what they are don't necessarily
             | coincide. E.g.: native americans are called "indians".
        
               | ben_w wrote:
               | A better etymological example would be that Stonehenge,
               | after which the category "henge" is named, isn't a henge.
               | 
               | (Aside from actual domain experts in this thread saying
               | it is an infection, that is).
        
               | jjk166 wrote:
               | But typically they do, and this is one of that vast
               | majority of cases.
        
             | ekianjo wrote:
             | I know, but it's a poor choice of word since it's nothing
             | like viruses or bacteria.
        
               | nkmnz wrote:
               | Viruses are nothing like bacteria, so it doesn't make any
               | sense to restrict the term of "infection" to those two.
        
             | knicholes wrote:
             | Contraction or portmanteau?
        
               | pygy_ wrote:
               | Contraction :
               | 
               |  _A portmanteau word is similar to a contraction, but
               | contractions are formed from words that would otherwise
               | appear together in sequence, such as do and not to make
               | don 't, whereas a portmanteau is formed by combining two
               | or more existing words that all relate to a single
               | concept._
               | 
               | https://en.wikipedia.org/wiki/Portmanteau
               | 
               | "Motel" and "fog" are portmanteaus, because you wouldn't
               | put "motor hotel" or "smoke fog" in sentences that accept
               | the accretions, whereas you could swap "prion" for
               | "protein infection".
        
           | dnautics wrote:
           | It's a self-replicating protein fold. Infection is correct.
           | (I Did prion research in grad school)
        
           | nabla9 wrote:
           | Infect is exactly the right word.
           | 
           | Infectious disease is also called transmissible diseases.
           | Infectious microorganism or agent can be a virus, bacterium,
           | protozoan, prion, viroid, or fungus.
        
             | webmaven wrote:
             | Don't forget memes. Not the current pop-culture meaning of
             | the term, but the original Dawkins version, which stretches
             | to cover things like mass-hysteria.
        
         | dukeofdoom wrote:
         | It seems like gain of function research is going on all over
         | the place. Maybe Alex Jones isn't total conspiracy nut, as the
         | media portrays him. He's just a head of what is commonly
         | disseminated. I watched an interview with him where he claims
         | to read a lot of research papers daily. Literally a dystopian
         | science fiction fantasy of a virus man made in a lab infecting
         | the world has a good likely hood of being true.
        
         | disabled wrote:
         | Keep in mind that the researcher was only 23 years old at the
         | time when the lab accident happened. In some ways, she was
         | basically a kid.
         | 
         | A human has a fully developed brain around age 25, but for some
         | people it is not until age 30 [1].
         | 
         | [1] https://bigthink.com/mind-brain/adult-
         | brain?rebelltitem=1#re...
        
         | mberning wrote:
         | Also I would be fine with studying prions in general. But I
         | don't think creating new ones is such a great idea.
        
         | bamboo2 wrote:
         | " vCJD or "classic" CJD, which is not known to be caused by
         | prions from animals. Classic CJD strikes an estimated one
         | person per million. Some 80% of cases are sporadic, meaning
         | they have no known cause, "
         | 
         | Who knows really. 10 years is a long time to wait and worry,
         | but it can take up to 50 years to express itself. Just imagine
         | spending your whole life worrying about this.
        
           | teknopaul wrote:
           | https://en.m.wikipedia.org/wiki/Bovine_spongiform_encephalop.
           | ..
           | 
           | Wikipedia thinks otherwise.
           | 
           | The whole UK population was eating mad cow for years until
           | humans started dying.
           | 
           | It was well known in advance that this was a crazy callous
           | risk taken by uk.gov
           | 
           | Careful what you read, the UK spend a lot of money on
           | propaganda at the time (to support beef exports) until the
           | case was well and truly lost.
        
             | cstross wrote:
             | Bear in mind that eating muscle tissue from infected
             | animals is a much lower risk than innoculating yourself
             | directly with brain matter from an infected animal.
             | 
             | The prion protein is expressed in the central nervous
             | system, which isn't legal for human consumption. Also,
             | ingestion of meat exposes it to stomach acid and digestive
             | enzymes before it gets into the consumer's circulation --
             | enzymes that break down foreign proteins into (harmless)
             | amino acids.
             | 
             | In contrast, accidentally injecting yourself with material
             | from an infected brain is about the most direct possible
             | infection route.
        
             | MichaelZuo wrote:
             | Mammalian proteins don't differ from each other all that
             | much so it makes sense the prions of any other mammal could
             | potentially infect humans. Though the further away in the
             | evolutionary tree the less likely I imagine.
        
         | bvm wrote:
         | Disclaimer: I know nothing about prions.
         | 
         | I think there's a mistake in the article, her website goes into
         | more detail:
         | 
         | https://soutien-a-emilie0.webnode.fr/lhistoire/ (gtranslate)
         | 
         | > Emilie is therefore working that day on samples of mice
         | infected with strains of human prions. It is important to
         | stress here that these were human strains and not animal
         | strains, the species barrier no longer protecting, the
         | infectivity of the pathogens handled by Emilie was therefore
         | total.
        
           | Y_Y wrote:
           | > Emilie travaille donc ce jour la sur des echantillons de
           | souris infectees avec des souches de prions humaines. ll est
           | important de souligner ici qu'il s'agissait de souches
           | humaines et non de souches animales, la barriere d'espece ne
           | protegeant plus, l'infectiosite des pathogenes manipules par
           | Emilie etait donc totale.
           | 
           | (In case anyone else wanted to double-check the translation.)
        
         | ignoramous wrote:
         | There is more
         | 
         | > _In a 2011 paper, his team reported that prions can spread
         | through aerosols, at least in mice, which "may warrant re-
         | thinking on prion biosafety guidelines in research and
         | diagnostic laboratories," they wrote. Aguzzi says he was
         | "totally shocked" by the finding and introduced safety measures
         | to prevent aerosol spread at his own lab, but the paper drew
         | little attention elsewhere._
         | 
         | ...
         | 
         | > _The government inspectors ' report concluded that Jaumain's
         | accident was not unique, however. There had been at least 17
         | accidents among the 100 or so scientists and technicians in
         | France working with prions in the previous decade, five of whom
         | stabbed or cut themselves with contaminated syringes or blades.
         | Another technician at the same lab had a fingerprick accident
         | with prions in 2005, but has not developed vCJD symptoms so
         | far, Bensimhon says._
        
       | redleggedfrog wrote:
       | I feel for Emilie. Those must have been tough years.
        
       | yarg wrote:
       | Time to develop protocols with slice resistant gloves.
        
       | fsloth wrote:
       | Could a cure be to stop proteosynthesis of the prion-protein?
       | 
       | 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).
        
         | nlitened wrote:
         | > "This protein, in its healthy, properly-folded state, is, if
         | not trivial, relatively unimportant. Its complete loss is
         | certainly not catastrophic."
         | 
         | If I at my age know anything about how real world works,
         | absence of evidence is not an evidence of absence, and I can
         | bet that this will turn out to be complete bullshit, and it
         | will get known in the future that this protein is important.
        
         | jhgb wrote:
         | It seems to me that in an adult, it may already be too late.
         | How would you remove the existing protein?
        
           | subroutine wrote:
           | 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/introductio...
        
           | im3w1l wrote:
           | Maybe it would degrade after some time, or be cleaned up by
           | the body. At worst it should stop the disease progression.
        
       | opwieurposiu wrote:
       | 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
        
         | newsbinator wrote:
         | 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...
        
           | neolog wrote:
           | That's a nice mechanism but wouldn't we all have to rebase
           | from lucy?
        
         | galactus wrote:
         | We sterilize everyone but them?
        
       | dmix wrote:
       | 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 wrote:
         | The 'Radium Girls' cases!
         | https://en.wikipedia.org/wiki/Radium_Girls
         | 
         | "It is safe"...
        
       | wil421 wrote:
       | 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.
        
         | carlmr wrote:
         | Just don't use the same saw.
        
         | hammock wrote:
         | At the very least a tourniquet could be considered. It only
         | takes one protein to get through though.
        
         | 0xfaded wrote:
         | 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.
        
         | connorproctor wrote:
         | 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.
        
       | JoeCoo7 wrote:
       | 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 wrote:
         | > "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."
        
       | brohoolio wrote:
       | 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....
        
       | briantakita wrote:
       | 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 wrote:
         | Look up how fluorine, the "devil's chemical", was found and
         | isolated: 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.)
        
       | Reventlov wrote:
       | 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
       | 
       | [2]: https://blogs.scientificamerican.com/artful-amoeba/prions-
       | ar...
        
         | bb123 wrote:
         | 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.
        
           | curryst wrote:
           | 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.
        
           | [deleted]
        
         | rs999gti wrote:
         | > Basically, you get infected, you die.
         | 
         | Avoid cannibalism and you should be fine.
         | 
         | https://www.npr.org/sections/thesalt/2016/09/06/482952588/wh...
        
           | Johnny555 wrote:
           | And avoid being in the same room with someone that's been
           | infected:
           | 
           |  _In a 2011 paper, his team reported that prions can spread
           | through aerosols_
        
           | hammock wrote:
           | >Avoid cannibalism and you should be fine.
           | 
           | And animal brains, and the areas around experimental research
           | labs...
        
             | [deleted]
        
             | Marsymars wrote:
             | Also see dura mater grafts:
             | https://www.cdc.gov/mmwr/volumes/67/wr/mm6709a3.htm
        
           | M2Ys4U wrote:
           | Avoid eating British beef in the 1980s/1990s, too.
           | 
           | Apparently 1 in 2,000 people in the UK may be infected.[0]
           | 
           | We still don't know if there's going to be a second wave of
           | disease from infections from that time period due to genetic
           | factors influencing incubation of the disease.
           | 
           | This is why people who were in the UK for longer than a few
           | months during the 80s and 90s aren't allowed to give blood in
           | various places around the world.
           | 
           | [0] https://www.bbc.co.uk/news/health-24525584
        
             | anthk wrote:
             | I was there with the mad cow disease. Not in the UK, but in
             | Spain, as commerce inside the EU made people truly paranoid
             | on eating meat from "untrusted" sources.
             | 
             | Scary, really scary.
        
             | croes wrote:
             | And don't feed beef to cows.
        
             | [deleted]
        
             | yhoneycomb wrote:
             | Serious question should I be avoiding traveling to the UK
             | over this??
        
               | hef19898 wrote:
               | No, that was 20-30 years ago.
        
               | dangerbird2 wrote:
               | At this point, the best prevention is to avoid eating
               | anyone who lived in Britain 20-30 years ago
        
               | hef19898 wrote:
               | Hopefully that's something we can all agree upon!
        
               | heavenlyblue wrote:
               | You forgot anyone who ate anyone who lived in Britain
               | 20-30 years ago.
        
               | acjohnson55 wrote:
               | Nope, but do avoid eating any British people over the age
               | of 20.
        
           | AuryGlenz wrote:
           | The mediation HCG is processed from pregnant women's urine
           | and has been shown to potentially contain prions. I took it
           | for years. Yay.
           | 
           | The sad thing is there's a recombinant version that would be
           | safe, but it also costs thousands of dollars.
        
       | imglorp wrote:
       | > 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 wrote:
         | yes, bleach kills microorganisms by denaturing their proteins.
        
           | Scoundreller wrote:
           | And by oxidizing everything else. It's not denaturation
           | that's making my clothes white again.
        
         | meepmorp wrote:
         | Hypochlorous acid denatures proteins, so yeah.
        
           | DoingIsLearning wrote:
           | 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 wrote:
             | >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.
        
             | persedes wrote:
             | yeah I wouldn't count on prions being as easily denatured
             | as other proteins. E.g. mad cow disease spread because
             | prions are not killed by simply cooking / boiling it.
        
               | resoluteteeth wrote:
               | Is there some specific reason to think that prions are
               | harder to denature than other proteins? It seems like mad
               | cow disease could also spread simply because meat is not
               | normally cooked to ensure that 100% of the proteins in it
               | are denatured.
        
               | nebopolis wrote:
               | Prions are essentially already denatured/unfolded. They
               | are a more stable state of a normal protein that has
               | biological function, and they interact with the
               | "correctly" folded proteins to cause them to denature
               | into additional prions. Often denaturing a prion means
               | actually breaking apart the protein into fragments or
               | amino acids, because it is already at the most
               | stable/denatured state. This is also why they are so
               | persistent in nature and so hard to remove from surfaces.
               | Apparently gas plasma sterilization is a promising
               | possibility. (this seems like an ok overview of the
               | challenges: https://pubmed.ncbi.nlm.nih.gov/21271212/).
               | Current procedures include things like autoclaving in
               | 121C sodium hydroxide for 30 minutes. Definitely not
               | something that fully cooking will inactivate.
        
               | persedes wrote:
               | In addition prions seem to contain disulfide bonds, which
               | tend to make proteins even more stable. (RNAse for
               | example has them and is notoriously hard to get rid off
               | unless you treat it with essentially H2S).
               | 
               | I haven't found a source for that, but according to my
               | bio teacher mad cow disease started, because they decided
               | to lower the temperature to pretreat animal waste (such
               | as bones and brain matter...) which then ended up in what
               | cows get fed with. Wikipedia mentioned nowadays they
               | simply leave out the bones and brain from the food.
        
         | tedivm wrote:
         | Yeah, bleach does affect prions. Most of the studies I could
         | find were tested on metal, not just someone's thumb. It looks
         | bleach doesn't do a great job of penetrating tissue so the hope
         | is to destroy the prions before they get into the cut, which
         | seems more hopeful than effective to me.
        
         | MisterTea wrote:
         | > Does bleach even affect proteins? Isn't that protocol for an
         | organism you're afraid of?
         | 
         | Yes, bleach's direct action is attacking proteins:
         | 
         | https://phys.org/news/2008-11-household-bacteria.html
        
         | COGlory wrote:
         | >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.
         | 
         | https://www.nih.gov/news-events/news-releases/household-blea...
        
           | quenix wrote:
           | Holy shit, 5 minutes _soaked_? That 's intense
        
         | hallway_monitor wrote:
         | I was thinking what if she amputated her thumb immediately? It
         | seems like an extreme reaction, but could it have prevented her
         | infection and death?
        
           | subroutine wrote:
           | That'd be a bold move, considering (1) it may not have
           | helped, and (2) it is possible the thumb incident wasn't how
           | she got infected. It's possible she got infected some other
           | random day in the lab. Maybe she rubbed her eye while wearing
           | a contaminated glove, or breathed in brain flecks through a
           | poor fitting N95, while slicing a particularly dry
           | cryosample.
        
           | [deleted]
        
           | bencollier49 wrote:
           | Good luck getting _that_ research commissioned!
           | 
           | Interesting idea though, and really it'd be a pretty sensible
           | trade-off.
        
         | ta988 wrote:
         | Yes it does destroy them efficiently, it unfolds them (so no
         | more prion effect) and also aggregate them so they can't
         | circulate.
        
       | chrbarrol wrote:
       | 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)
        
         | Omavel wrote:
         | bio-terrorism is not effective.
        
           | tyingq wrote:
           | What's "effective" mean in this context? The anthrax letters
           | in the US in 2001 certainly dominated news cycles.
        
             | ekianjo wrote:
             | 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.
        
               | triceratops wrote:
               | "Terror" is the point of "terrorism". It's in the word.
        
               | inglor_cz wrote:
               | Point of terrorism is achieving a political change
               | through terror. Terror is not the end in itself.
               | 
               | From this point of view, mailing of anthrax in 2001 was
               | very inefficient.
        
               | jessaustin wrote:
               | We need to expand the set of possible actors and goals.
               | The anthrax campaign happened immediately after 9/11. The
               | result of the campaign was the end of effective political
               | opposition to the massive expansion of military and
               | "secret" aspects of the USA government made possible by
               | 9/11. _Cui bono?_ Certainly not the several implausible
               | patsies the authorities immediately had lined up to
               | blame.
               | 
               | Numerous USA calamities make a lot more sense when we
               | study them with an eye toward who benefits rather than
               | who is blamed.
        
               | ekianjo wrote:
               | Terror only propagates through mass media. Mass media is
               | the issue.
        
               | PKop wrote:
               | Given that an attack of this sort _would_ be propagated
               | through mass media, then the claim that the attack would
               | be ineffective is wrong.
        
               | emptyfile wrote:
               | >it's not effective in doing anything but news cycles.
               | 
               | That's literally the desired effect. What you're thiking
               | of is war.
        
               | ekianjo wrote:
               | 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.
        
               | tyingq wrote:
               | 5 envelopes, 5 dead, 17 more infected.
        
             | myWindoonn wrote:
             | The most effective bioterror attack in the USA was https://
             | en.wikipedia.org/wiki/1984_Rajneeshee_bioterror_atta... and
             | it killed zero people, affecting 751 people in total.
             | Bioterror is expensive and difficult.
             | 
             | For prions specifically, the main "problem" (which is a
             | good thing overall for us) is that they're naked proteins,
             | and they denature relatively quickly when outside of a
             | protective environment. Spray them onto crops? Crops and
             | soil are covered in small bugs and microbes which break
             | down anything too long, including proteins and carbohydrate
             | chains. No, in order to have prions in the food supply, you
             | need something like cannabalistic cow-raising practices or
             | other ways of recycling prions in a wet+hot safe
             | environment.
        
               | Apocryphon wrote:
               | So you just inject them into the food supply?
        
               | Johnny555 wrote:
               | Can't you just spray them into a hamburger meat grinder
               | at a meat packing plant to protect the prions in beef?
               | But it'd be a pretty ineffective terror attack if it
               | takes 10 years for people to show symptoms... though that
               | could also mean that attack was already made 5 years ago
               | and we just don't know yet.
        
               | Marsymars wrote:
               | > For prions specifically, the main "problem" (which is a
               | good thing overall for us) is that they're naked
               | proteins, and they denature relatively quickly when
               | outside of a protective environment.
               | 
               | How does this jive with the seemingly extreme difficulty
               | of effective sterilization of prion-contaminated objects?
               | 
               | https://academic.oup.com/cid/article/32/9/1348/291736
        
           | nabla9 wrote:
           | Terrorism that is effective is usually called warfare,
           | genocide or something else.
        
             | natmaka wrote:
             | Indeed. http://exiledonline.com/wn-38-ira-vs-al-qaeda-i-
             | was-wrong/
        
         | ekianjo wrote:
         | if you want to kill lots people thats pretty much the worst way
         | of doing it.
        
           | mschuster91 wrote:
           | The definition of terrorism is "inciting fear", not
           | necessarily "kill thousands". A million people afraid if they
           | caught BSE/cow madness is _definitely_ hitting that
           | definition, especially as the stuff can 't be tested for...
           | people exposed to prions will have that sword hanging over
           | their neck for the rest of their lives, and the affected area
           | will be out of order for _years_.
           | 
           | To this day, many countries ban all people who lived in the
           | UK between 1980-1996 from donating blood
           | (https://www.srf.ch/news/schweiz/bse-nachwirkungen-blut-
           | spend...) simply because of that fear.
        
         | overlordalex wrote:
         | I've always thought a _very_ effective strategy would be to get
         | a crop-duster and spray a prion-contaminated mist over a major
         | city, basically contaminating every public surface in a large
         | area
         | 
         | I'm also not sure how you would begin to clean up afterwards -
         | do you remove the surface of everything in your city?
         | 
         | To those saying bio-terrorism is ineffective or would not kill
         | a lot of people in any appreciable time-scale, think about the
         | terrorism aspect - imagine not being able to interact with any
         | surface? It would be like a permanent covid lockdown on the
         | area
         | 
         | The psychological effect of a large-scale area-denial would be
         | massive
        
           | dnautics wrote:
           | It's not really effective because it takes years to manifest
           | symptoms of prion disease. Terrorism usually has a message
           | attached to it, and "you're gonna start dying in 10 years" is
           | not conducive to messaging.
        
             | chrbarrol wrote:
             | It is true that hiding it for many years would make it less
             | attractive for terrorists, however one could imagine
             | another strategy where you wait 12 months before
             | anonymously telling the authorities you did it, without
             | telling them how. (While threatening to do it again)
             | 
             | Having some unknown person(s) spreading Prion diseases
             | around that cannot be detected until way after it happens
             | would be close to the perfect terrorist attack imo. You
             | could probably keep the attack going for at least a year
             | before authorities get their act together, all the while
             | people would be scared shitless as it would be very hard to
             | know how the original group got infected a year earlier if
             | you choose the right product to contaminate. (Not even
             | mentioning the difficulty finding who did the contamination
             | even if the specific product is found)
        
               | XorNot wrote:
               | Authorities are really good at de-anonymizing anonymous
               | messengers. Who have no incentive to spread an unverified
               | rumor they can't prove.
        
             | medstrom wrote:
             | In this case I guess the message targets people in other
             | cities than the doomed one. "This could happen to your city
             | too".
        
               | dnautics wrote:
               | you'd still have to wait a decade after the first attack
               | to make threats on the second city.
        
               | medstrom wrote:
               | I think reading about prions is the scary part, no need
               | to wait for the deaths.
        
               | dnautics wrote:
               | feel free to experiment with terrorism establishing
               | credibility using a different psychological model. I,
               | personally, won't.
        
               | Apocryphon wrote:
               | Why even bother with terrorism? It sounds like a
               | potentially gruesome and sadistic weapon with which to
               | use against a particularly bitter foe- the terror is only
               | incidental. Maybe it's akin to using a dirty bomb.
        
               | BoxOfRain wrote:
               | Wouldn't an entire city being sprayed with prions be a
               | fairly apocalyptic event in and of itself? Those prions
               | aren't going to go away, they'll get blown about and
               | distributed by the millions of cars, buses, and trains
               | going in and out of the city. What can you realistically
               | do about that, go full Imperial Inquisition and raze the
               | entire section of the country to the ground?
        
             | da_big_ghey wrote:
             | living with knowing that i probably have 10 yrs before die
             | in a gruesom neurodegeneration, knowing that if i interact
             | much with our world that maybe i spread this to others,
             | seriously terrifying, depressing, would induce misery.
        
               | dnautics wrote:
               | I'm not disputing that. (also, prions aren't really
               | human-to-human contagious, unless you're into eating your
               | friends' brains or swapping syringes)
        
       | dekhn wrote:
       | 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 wrote:
         | Makes complete sense that there was resistance until a viable
         | mechanism was proposed. Otherwise it just sounds like voodoo.
        
           | boxed wrote:
           | Not really. Look at how Darwins theory of evolution was
           | published in 1859, while the mechanism wasn't really clear
           | until at earliest 1928, and in some ways it's still not fully
           | understood.
        
           | dekhn wrote:
           | 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 wrote:
             | 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 wrote:
               | 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.
        
               | jjk166 wrote:
               | Clearly you do not need to know the mechanism by which
               | light waves propagate to prove that light waves do
               | propagate. It would be ass-backwards to wait until
               | special relativity was formulated to accept the existence
               | of electromagnetic waves.
        
           | ricksunny wrote:
           | If ancient Romans could have shown correlation & causation
           | between lead pipes & drinking vessels to poor health
           | outcomes, they should have ceased using lead for these
           | purposes even while lacking the capability of understanding
           | the underlying mechanism. There's no voodoo in that.
        
           | contravariant wrote:
           | It's not exactly that rare for a phase transition out of a
           | metastable state to happen spontaneously when given an
           | appropriate nucleation seed.
        
         | dmix wrote:
         | This bit in the article drove home how difficult it would be:
         | 
         | > The two types of CJD can only be distinguished through a
         | postmortem examination of brain tissue.
        
         | varispeed wrote:
         | Is it because many researchers put their ego first over the
         | science? It's so many times that they ridicule ideas, that
         | often amounts to bullying and then they rarely even say sorry
         | when it turns out they were wrong. Why won't academia flush out
         | these types of people? The toxic atmosphere with no way of
         | getting rid of bad apples is what put me off from studying.
        
           | raincom wrote:
           | This phenomenon is studied in the history and philosophy of
           | sciences; it is called "paradigm change" ala Thomas Kuhn.
           | Yes, researchers who work in the dominant 'paradigm' tie
           | their psychological identities with the paradigm they are
           | working on. So, this resistance is expected.
           | 
           | 'How to overcome resistance' is studied in Sociology of
           | Science.
        
           | dekhn wrote:
           | it's nuanced. prusiner himself was a bully who ran a toxic
           | lab. my group collaborated with him and we got an eyeful.
        
           | hprotagonist wrote:
           | >Why won't academia flush out these types of people?
           | 
           | Because this behavior is part of human nature. Do you think
           | scientists as a class are are somehow exempt from this, say,
           | by some form of rationalistic purity?
        
           | Swizec wrote:
           | It's because discovering something completely new and unheard
           | of that isn't even hinted at hy any existing theory is
           | extremely rare. Most people with such claims turn out to be
           | wrong, doing bad experiments, fudging data, or worse.
           | 
           | The scientific community is resistent to such things because
           | 90% of the time they are right to be resistant, the
           | hypothesis is conclusively disproven, and we never hear about
           | it again.
           | 
           | Sometimes, rarely, the claim comes through, survives the
           | scrutiny, and scientists quickly change their minds based on
           | new established fact.
           | 
           | Imagine if someone came out with "I have found a way to run
           | 50 compute cycles per CPU instruction!!". We would
           | justifiably ridicule that person's idea as implausible or
           | impossible. It would take a lot of evidence to change the
           | whole computer industry's opinion on what's possible.
        
         | agumonkey wrote:
         | any article about the replication mecanism ? not too short, not
         | too detailed (if possible) thanks
        
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