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Science

France Issues Moratorium on Prion Research After Fatal Brain Disease Strikes Two Lab Workers (sciencemag.org) 105

Five public research institutions in France have imposed a 3-month moratorium on the study of prions -- a class of misfolding, infectious proteins that cause fatal brain diseases -- after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. From a report: An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job. If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Emilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.
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France Issues Moratorium on Prion Research After Fatal Brain Disease Strikes Two Lab Workers

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  • Has anyone checked if they visited Fort Detrick?

  • ... would be a whole new nasty type of disease. They're not alive so you can't kill them, you can only destroy them with strong bleach or similar. Lets hope it requires long term exposure to cause this and isn't a case of a single molecule can trigger it.

    • by Anonymous Coward

      What do you mean "whole new"? Where you alive during Mad Cow Disease? What do you think it was?

    • by Tablizer ( 95088 )

      They are like super-simple viruses. Many suspect the boundary between "virus" and "prion" is fuzzy such that there may be undiscovered "organisms" anywhere in between virus and prion.

      • by Entrope ( 68843 )

        Prions are proteins, with no genetic material of any kind. Exactly what continuum do "many" see between them and even RNA viruses?

      • They are like super-simple viruses. Many suspect the boundary between "virus" and "prion" is fuzzy

        No. Viruses and prions are completely different. There is no overlap at all. Prions are proteins. Viruses are based on RNA or DNA.

        A virus has more in common with a chimpanzee than it does with a prion.

        • by Tablizer ( 95088 )

          > Prions are proteins. Viruses are based on RNA or DNA.

          RNA and DNA are also proteins.

          > A virus has more in common with a chimpanzee than it does with a prion.

          Viruses only carry a small portion of DNA, say 1/5000th of an (altered) chimp. Prions and viruses both hijack the machinery of the host to reproduce. Prions just take a simpler route.

          • RNA and DNA are also proteins.

            They aren't. They are the patterns that proteins are made out of (protein encoding patterns).

            DNA/RNA - made of nucleotides, which have a common base (a phosphate group linked to a five-carbon sugar), with a differentiator attached.

            Protein - a structure made of amino acids

            Amino acid - A carbon cored base (C + COOH + HN2) with a differentiating carbon chain which is different in each amino acid.

    • From what I remember, prions cannot be disinfected like viruses and bacteria with conventional chemicals. One method was high heat (fire) or very strong chemicals which destroys plastics.
      • Re: (Score:2, Informative)

        by iggymanz ( 596061 )

        Not true, prions are quite fragile outside the body like any other isolated protein if you're talking about chemicals, bleach can denature them for example.

        Inside the body is another matter, no Trump Chlorox Cocktail can cure it.

        https://www.nih.gov/news-event... [nih.gov]

    • by hey! ( 33014 ) on Wednesday July 28, 2021 @12:46PM (#61630469) Homepage Journal

      It's theoretically possible to target prions with vaccines that generate antibodies to protein sequences displayed on the prion but not the normal form of the protein. This doesn't mean it would always be possible in practice.

    • by ceoyoyo ( 59147 )

      All the other nasty types of infectious disease are made of proteins. Prions are just the atomic unit of infectious disease. Being the atomic unit, their options for adaptation are basically nil, unlike everything more complicated.

  • I found a guy claiming prions aren’t dangerous at all! Fire all those scientists and put them in jail for flip flopping. As a matter of fact let’s cancel Copernicus for flip flopping on the earth being the center of the universe.

  • by Cy Guy ( 56083 ) * on Wednesday July 28, 2021 @12:19PM (#61630315) Homepage Journal
    A prion disease that can be contracted by a simple pin-prick might explain the brain disease now being found in New Brunswick, Canada. https://www.nytimes.com/2021/06/04/world/canada/canada-brain-disease-mystery.html

    (also - this is terrifying!)
  • I think this means I can fill in a square on my 2021 Disaster Bingo card. Pretty sure I had "Prion Plague" on there.
    • by sinij ( 911942 )
      It is still not too late in the year for Alien Invasion.
      • Biden caused that, done. Then appointed smiling dingbat VP to be czar of issue.

      • by Anonymous Coward

        All I need now is "your planet is in imminent danger of being eaten by an enormous mutant star goat".

    • My card has WW III square, maybe Israel can get in Russian face a little more regarding Syria to get that party started

  • It sounds like they had sufficient protocols in place but that they weren't followed. That's always a bad time in the lab.
    • by sinij ( 911942 )
      What protocol is 100% followed all the time by everyone forever? Even if you write "don't stick you dick into running meat-grinder" safety protocol, someone somewhere would still end up mangled.

      If you play with shit that could potentially kill humanity if it escapes a) don't do it, b) if a is not an option, do it on a desert island.
      • It was a needle stick; its an exposure, not a release. And they had mitigating protocols in place that weren't followed. This is why people try to promote 'safety culture' since it is a good way to avoid ending up dead.
      • Lots of people have died in lab accidents including chemical, nuclear and biological. I don't see anything special about this issue with prions, unless people were spreading it to others.
        • The problem is that a chemical or nuclear lab accident would be limited locally to whatever city/country/region had the lab. In a global world, a biological threat could spread throughout the entire world before we have even noticed the leak. Case in point with the 'rona, a guy coughed on the soup in China and the rest of the world found themselves out of jobs for years.

          • Prion diseases really aren't contagious in the sense that respiratory viruses are. To get it from another human being, you'd have to eat their brain (see "kuru") or inject the proteins into your body with a needle like the unfortunate researcher.

      • Mad cow and similar can't "potentially kill all humanity if it escapes", stuff has been around for at least centuries and more like from before the dawn of humans.

        Animals go and come from desert islands too, in surprising ways besides the obvious ways.

        • by ceoyoyo ( 59147 )

          Well before humans. Prions are misfolded proteins that are more stable than their properly folded versions. They're as old as proteins themselves.

  • Do they do gain of function research on prions?

    On the hand it would be criminally insane, on the other these are infectious disease scientists so not entirely unlikely.

    • > On the hand it would be criminally insane, on the other these are infectious disease scientists so not entirely unlikely.

      If a strong enough argument is made, we can do GoF and minimize risk.

      1. Antarctica only. Build a lovely facility there. Power it with a fission reactor of the type used by Navy ships. Domes with gardens and all. It doesn't have to suck.

      2. Five Navy quarantine ships are required. Three in use at any given time. One headed South, two headed North. Each Southbound passenger spen

      • And then you fall on your knees and cry at the hubris of man, as you realize that your Draconian measures were precisely the reason that that promising, young scientist turned evil and intentionally created a super-weapon to destroy humanity.

      • 9. GoF is banned on the other six continents. Violating the protocols must be treated as a presumptive War Crime.

        I read "golf is banned", and was fully on board. %^)

    • Gain of function, the new phrase parroted by Fox News viewers. I'm sure Fauci will get blamed for this somehow.

      So now we have: ...But her emails! ...But Benghazi! ...But his laptop! ...But gain of function!

    • It'd be like doing GoF on table salt, the question doesn't make sense. I suppose you could use a protein folding simulator to make novel prions?
      • You can create various forms of prion protein with plain old DNA/RNA techniques, expose them to varies forms of scrapie and get whole new forms of scrapie.

        That's how we got in this mess. Eating sheep brain with scrapies did not make humans ill, but then we fed sheep brains to cows and we got CJD.

  • ... if this has any commonality with that story from May this year [slashdot.org] about a mystery brain disease in Canada?
  • CJD sometimes occurs "sporadically" - a protein mis-folds inside the body randomly and starts being replicated.

    It can also occur genetically, with a really tragic combination of genes.

    According to an EU study [wiley.com] referenced in the CJD Wikipedia article, 87% of cases they studied were sporadic, 8% genetic, 5% iatrogenic.

    Prions are much simpler than viruses - it would be a lot harder to find differences and track them back to a source. So, from a causality point of view, you're left with guessing.

  • There is no doubt they got infected at the market eating bat. No investigation needed

  • In June 2019, an INRAE lab worker named Emilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life;

    This is total BS. Anybody that is working in these arenas KNOW the possible threat to themselves. When I was working at a lab at CDC, we were required to take experimental live vaccines for West Nile, Encephalitis, and Dengue. We did this KNOWING that it could kill us, or worst yet, brain death. In addition, I had a lab accident where a mag stirrer broke a beaker. I was not paying attention and put my face down close to it to see why it was broken. But, that beaker was the radiotracer live virus that we w

  • CJD has zombie like symptoms.

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