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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.



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.


> Emilie travaille donc ce jour là sur des échantillons de souris infectées 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 barrière d'espèce ne protégeant plus, l'infectiosité des pathogènes manipulés par Emilie était donc totale.

(In case anyone else wanted to double-check the translation.)


It's been a bit since I took french but that looks pretty good to me.


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.


We know that aerosoled brains of animals can cause disease in humans.

Take a look at this: https://en.m.wikipedia.org/wiki/Progressive_inflammatory_neu...

This disease was discovered due to an alert translator (most likely translating from Spanish to English) recalling that he had seen patients’ records before that had the nearly the same exact history.

After investigation, it was discovered the patients worked in a slaughterhouse near the pig brain section, where the brains were being aerosolized. Many ended up developing a progressive inflammatory neuropathy.


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. As everyone knows, she ended up dying because of this.

A human has a fully developed adult 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...


I used to mention this to my binge drinking buddies in college when they wanted me to kill more than a few brain cells with them. In general moderation in all things.


I wonder how much time you would have to sever your finger if you get poked by a tainted forcep?


That’s beyond the point. I am saying this as somebody with type 1 diabetes who has to poke themself with needles to draw blood daily.

Her finger most likely got lanced, by a very thin forcep tip. It was never about “severing”.


I think the point was, if you were injected with the prion in a finger, how long would you have to sever that finger to prevent the prion from entering the rest of your body.


Can we call this the "World War Z Problem"?


I think this article, or another, said that probably applying disinfectant immediately would've been enough.


Prions are not affected by disinfectant


You think you know more about chemistry than...a lawyer?

"The thumb should have been soaked in a bleach solution immediately, which did not happen, [the family's lawyer] adds"


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.


“ 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.


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.


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.


>the central nervous system, which isn't legal for human consumption.

Source? I've seen calf brain on the menu plenty of times.


It was banned in the UK almost as soon as BSE was identified as a prion disease. Even before they culled the entire beef herd.

(Source: I live here, I'm old enough to remember it dominating the news cycle for months.)


All sales of beef on the bone was banned too. Even Bovril had to change their recipe for a few years.

The ban was introduced on December 16, 1997, after government advisers reported small risks that small nerve endings near beef bones and bone marrow might be infective. The ban included cuts such rib roasts and oxtail, as well as soups and stock cubes made in Britain from beef bones.

The ban was lifted Tue 29 Nov 1999, but other bans on using as food more risky parts of cattle - brain, eyes, tonsils, spinal cord, spleen and intestines - remained in force, as did ban on use of bones in manufactured food and cattle more than 30 months old are banned from the food chain.

Things have been eased further since 1999 but I'm not sure we can get brain on the menu in the UK...


Prions are pretty robust…


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.


Aren't some forms of it contaigous if you come in contact with malformed prions?

Making it a inheritable desease that can afflict others?


Actually, this is poor skimming on your part.

Émilie Jaumain - the person refered to in your first quoted paragraph - worked at INRAE and the lab there works on prions that can infect humans. She cut her finger, and became infected.

Adriano Aguzzi's lab at the University of Zurich is the one that only works on mouse-adapted sheep prions. Presumably a similar accident wouldn't be possible there.


Also I would be fine with studying prions in general. But I don’t think creating new ones is such a great idea.


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.


You are conflating lab leak with intentionally crafted bioweapon.


> You are conflating lab leak with intentionally crafted bioweapon.

> intentionally crafted bioweapon

they didn't mention anything about that anywhere.


Is he? He only talked about lab leaks. You brought up the nonsense bioweapon theory.


But then he goes to the HN endorsed point of "lab leak" hypothesis.


A bioweapon that can't be targeted doesn't seem very useful, does it?


Could easily be both. Bioweapon development that was accidentally leaked.


“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.


There is a difference between "never been shown to" and "shown to never".


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_(philosophy)#P...

[1] https://en.wikipedia.org/wiki/Black_swan_theory


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"


>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?


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.


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."


That was meant to be my point, the down vote makes me think I didn’t make it clearly enough…


"There is no evidence that..." is my recent favorite.


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.


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.


"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.


It's a self-replicating protein fold. Infection is correct. (I Did prion research in grad school)


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').


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")


Contraction or portmanteau?


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".


typo -

    "Motel" and "fog" are portmanteaus, because you 
    wouldn't put "motor hotel" or "smoke fog"
s/b

    "Motel" and "smog" are portmanteaus, because you 
    wouldn't put "motor hotel" or "smoke fog"


What things are named and what they are don't necessarily coincide. E.g.: native americans are called "indians".


But typically they do, and this is one of that vast majority of cases.


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).


I know, but it's a poor choice of word since it's nothing like viruses or bacteria.


Viruses are nothing like bacteria, so it doesn’t make any sense to restrict the term of “infection” to those two.


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?


I'd say so.


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.


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.


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.


Don't prions reproduce by re-folding other proteins to match themselves?


they dont replicate.


Actually, they do. They refold normal proteins into more versions of themselves. I went and checked after this.

https://en.wikipedia.org/wiki/Prion#Prion_replication

There is a complex dependence on forming fibres that makes them slow (long lead time to symptoms) and then fast (dead soon after getting sick).


Infection means contamination / spoilage.

https://www.etymonline.com/word/infection


Prions contaminate


I'm just saying it does reproduce (or replicate to use the exact same word op used). Whether it's correct to use any of the other terms I will leave to others


Infect is definitely the right word here.




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