As far as I know, all the prion diseases are actually in the same protein. Different misfolds, and different sources, but all related to a single protein.
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I almost mentioned the mystery disease that Canada is dealing with at a small community. They’re not sure if its related to prions yet but its certainly still an awful thing that the residents of that area have been suffering.
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That*s why I think in terms of it being a computer glich, being a programmer. It’s not understood all that well, is less of a reproduction error than a crash, the fold going wrong for some reason. Which causes a nearby protein to crash as well. More like bit rot than a bug. No malicious agent behind it.
Again, it’s because I deal with persnickety software that I use bad analogies that make it easier for me to relate to.
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I dunno about that…
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Hit up your bookstore and grab a book called Deadly Feasts.
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Proteins are interesting because in traditional chemistry terms they are very easy to characterise – just a long, specific sequence of 39 or so simple units – but their actual behavior is a matter of how that chain naturally tangles up at much larger scales, which is determined by their environment as much as the DNA that codes for them. It’s kind of like, a crochet hat is literally nothing more than a piece of yarn – if you pull on the loose end you can straighten it out – but understanding that piece of yarn tells you nothing about the hat, and the same piece of yarn could equally be a scarf or an amigurumi C-3PO doll, depending only on the history of what happened to it.
Except protein chains are kinked and curled in such a way that, when jangled around in the right context, they knot themselves into particular shapes. Where that context includes other existing proteins, it’s easy to see on a hand-waving level how one misfolded protein can cause others to conform themselves the wrong way, particularly where a chiral center is inverted. You can imagine a Bob Fosse dance routine where one dancer mistakenly crosses their left leg over the right instead of vice versa, forcing all the other dancers in the line to do the same, and then they just carry on with the choreo reversed, until at some point they’re supposed to cross with another line of dancers and it turns into a big pileup.
That is to say, it’s not mysterious in principle how “correct” proteins could infect each other with defective conformations; it’s just that no one has worked out how to reason adroitly about protein folding at the enormous scales involved.
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all the nate silver approved burrito places in frisco had cow brain (“Sesos”) but I was never brave enough to try… maybe that was a good thing
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Some people (like myself) have wondered if normal Alzheimer’s or dementia is the result of eating meat from a national supply that is more heavily prion-infected than we’d ever admit. Instead of 10 years incubation for Mad Cow, the Alzheimer’s prions take 30, 40, 50 years…
If that is true we would probably see, at some point, a fairly drastic reduction in the prevalence of Alzheimer’s since the cattle re-feeding cycle was mostly ended in the late 1990s. But prior to any decrease the numbers would be expected to increase (like they are) since meat-and-bone-meal feeding was becoming more prevalent over time until it was discontinued in a hurry.
Yet another internet armchair theory for y’all to mull over, with some just-knowledgeable-enough-to-be-dangerous circumstancial evidence.
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If there was ever an explanation for the adaptive reason for why most organisms are seemingly programmed for death and cultures optimize for quick decomposition (along with instinct for revulsion towards cannibalism), it’s this for me. Death and burial (or cremation) is the long-held barricade quarantining prions.
Any species (or even local ecosystem) that played with extra long life got overrun by prions and got wiped out.
We’ve messed this natural cycle up royally. And though torn, I think we do need to stop playing with prions in the lab. Even if we can figure out the perfect enzyme to properly refold a protein that’s crumped into a prion, there’s gonna be more that escapes that one-off solution. Better to just keep it from spreading.
South Korea might end up paying for its “clever” program of taking food waste and turning it into animal feed.
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Yeah, about that…
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Keep in mind too that it will disqualify you from organ donation as well.
One of my friends who was with me in study abroad brought that up to me just last year.
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Oh, I was donating blood and was being grilled on whether I was in certain countries in Europe for 6 months during the 90s and early 2000s. I guess it was about that!
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Eye Prions? Transmitted via optometrist equipment? I did not need to read that right now… I’m a year overdue for my next appointment already…
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I once made a list of things that have landed on my safety glasses: plastic shards (wedged into the lens), diesel, fire (singed the eyebrows)… Yeah, I’m glad I wore them.
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I remember the news reports from 1997 about 11 cases of skwirl-brain-related brain-rot deaths in Kentucky. So I tracked down the original paper and the authors only cited 5 cases. Is there more recent research?
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Exact same boat here. Spent most of a year in North Yorkshire in '85/'86. I gave blood regularly until I couldn’t. I check periodically to see if the restriction has lifted and am usually relieved to find it hasn’t been. My wife gives enough for the both of us and hers is better than mine.
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Crap. I’ve never before heard this and currently have the organ donor box checked on my license. My family is also informed of my wishes. I wonder if I should change my status, as nobody will be able to ask about my time in Europe at the, ahem, relevant moment.
(edited to correct typo)
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I am an RN and worked on a medical neurology unit in Chicago in the early 1980s. I took care of a patient with Creutzfeldt-Jakob syndrome, caused by prions. This microorganism was understood to be so transmissable that even a piece of one could cause this horrific disease that always results in death. Word was many pathologists would not autopsy patients who died of it, since they were concerned they could get it from the body.
We need substantially better protection for workers that do research and for those who provide direct care to these unfortunate people. Otherwise, we should stop handling these bodies and cremate them for the public good.
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I was just reading up on current guidance and it appears that the Red Cross made changes last year. Specifically, they seem to have lifted restrictions on donations from folks who spent time at European posts (who apparently sourced lots of British beef during the key time frame) based on this guidance from the FDA.
Folks who lived in the UK (raises hand) are still excluded.
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