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I understand prion diseases are rare and it's more likely I'll die in a car accident or something, but the persistence of prions is just a horrifying concept.

• rch 9y ago
  I'll probably pass on the Elk jerky on my next road trip through SW Colorado.
• nathancahill 9y ago
  It can happen anywhere at anytime, that's why it's so terrifying.
  • Florin_Andrei 9y ago
    There's a bit of good news in the fact that the biosphere has not only endured these last 3.8 billion years or so, but it has thrived. So there's probably no prion apocalypse in the future - at least not at the scale of the whole biosphere. Individuals and species, on the other hand, seem to be fair game.
• quantumhobbit 9y ago
  What would a cure for prion diseases even look like? Are prions targeted by the immune system at all? That to me is what is scary about them.
  • itchyouch 9y ago
    From what I recall, they are seen as normal proteins by the immune system, thus not targeted. A simple illustration on prions was to imagine it like a busted cable end that would break the socket it plugs into, then the socket would break other cable ends plugged into it. This would occur until all sockets and cable ends were broken in the same way.
    • phkahler 9y ago
      Decades ago, we had a PCMCIA card that did exactly that. A bent pin distorted the plastic in the socket and that led to more bent pins. Of course you had to try to force them in to propagate the problem and we never tested to see how many generations it could last. Once discovered we threw out all defective hardware which was just a few cards by then.
      • jacobush 9y ago
        I have heard this one before!
    • jandrese 9y ago
      It's the Zip Drive click of death for your body.
      • LordKano 9y ago
        I was going to make this same point.
  • abetusk 9y ago
    Not a direct answer to your question (because I don't know) but there's a couple doing research into a cure for prion disease because one of them (Sonia Vallabh) has it. Eric Minikel has been blogging about their research [1] [2], maintains an active Twitter presence [3] and has GitHub [4] repositories on the issue. The blog has a lot of information for anyone wanting to know more about bioinformatics, basic research and prion disease in particular. [1] http://www.cureffi.org/ http://www.cureffi.org/ [2] http://www.cureffi.org/about/ http://www.cureffi.org/about/ [3] https://twitter.com/cureffi https://twitter.com/cureffi [4] https://github.com/ericminikel https://github.com/ericminikel
    • dmix 9y ago
      > Sonia and I set out on a quest to re-train ourselves as scientists. We both started taking night classes, reading papers, calling up scientists, going to conferences. We left our old careers and found jobs in research labs, and eventually enrolled at Harvard Medical School, where we are now PhD students in biological and biomedical sciences. Wow, what a great story. Inspiring that you can change careers mid life and be successful. Thanks for sharing.
  • dllthomas 9y ago
    > What would a cure for prion diseases even look like? Well, I mean, in some way the prion behaves differently than its normal counterpart. A molecule that interacted with that in such a way as to destroy the prion would seem to be a cure, right? At least, if not otherwise too harmful...
    • s0rce 9y ago
      Or bind with it such that it is now inert, refolded back to a proper configuration or label it for destruction by the immune system.
    • amelius 9y ago
      Or how about a molecule that binds to the harmful part of the prion?
  • dnautics 9y ago
    If we do devlop a cure for prion diseases, short of a biofilter in a transporter beam, it will probably be some sort of directed antibody that magically only recognizes prion form of the prion protein, sequesters it and prevents spread; and maybe if we're clever at molecular engineering, also recruits the body's natural capability to clear these proteins (insulin-degrading enzyme).
  • scythe 9y ago
    There are molecules that cause prions to "refold" back into the healthy form. The problem is that so far every such known molecule is highly toxic, e.g. guanidinium chloride.
  • dragonwriter 9y ago
    > What would a cure for prion diseases even look like? You'd need something that responded to the physical conformation difference in the prion (which is clearly possible because prion disease is exactly a difference in behavior based on that) in a way which somehow selectively disrupts or expels the prion form of the protein (while different reaction is clearly possible, the desired difference is less clearly acheivable.) I suppose some kind of nanomachine that actually physically reconfigured proteins from the prion conformation to the healthy one might also be plausible, eventually.
    • ajnin 9y ago
      The particularity of prions is that when they come into contact with the normal form of the protein, they can change its conformation to create a new prion (that's how the disease spreads). In that light I think an "anti-prion" protein should be possible, going around the body converting prions back into healthy molecules.
  • Cybiote 9y ago
    We can look at kuru survivors who sadly, recently underwent rapid natural selection. Among this population are those possessing a variant of the prion protein gene that conveys strong resistance to prion diseases and the propogation of misfolded prion proteins. Here's an article: http://www.nature.com/articles/srep21804 http://www.nature.com/articles/srep21804
• Razengan 9y ago
  > Basically if you've got a prion disease it's just a matter of time before they end you. Unless you had a mind backup and a second body ready. Which, I think, all medicine ultimately aims, should aim, for; Why bother learning how to repair the single same body over and over (which won't help in unexpected catastrophic failures aka fatal accidents anyway) when you can just grow and jump into a new one?
  • snowwrestler 9y ago
    While we have thousands of years of cultural belief that mind and body are distinct, I'm not aware of any scientific evidence for that. And there is a lot of evidence that the mind is directly dependent on the state of the body--change the body (drugs, injury, etc) and inevitably the state of mind changes too. So I don't know of any reason to think that it's even possible to separate a mind from a body, let alone move one to a new one.
    • Razengan 9y ago
      Just copy everything then. There's no reason to roll over and just accept death from aging, accident or violence, any more than we accept death by flu or any other curable disease. Things which would've outright killed people 200 years ago merely cause a few days of a runny nose to us, so to speak. You cannot deny that medicine is, after all, basically the quest for immortality, and that is where it's going to end up.
      • scythe 9y ago
        You cannot copy "all" of the information stored in the human body. Briefly, this happens because the physical limitations of information storage require cooling (Landauer's principle) which requires kT×log(2) energy per bit, or about 2.5 zJ at room temperature. The brain's memory capacity is about 2.5 * 10^15 bytes (https://www.scientificamerican.com/article/what-is-the-memory-capacity/ https://www.scientificamerican.com/article/what-is-the-memor...), but the amount of information which can be retrieved is much smaller than the amount of information required to fully describe the system -- in the same way that knowing the contents of my hard disk will not help you recreate my laptop. The brain weighs about 1300 grams, of which maybe 5%, or 65 grams, is protein, composed of amino acids which weigh about 0.25 zeptograms, so contains about 2.6 * 10^23 amino acids. If we assume that we need a kilobit of information per amino acid, which seems like a lowball, it would require about 650 kilojoules of energy at the absolute thermodynamic minimum (vastly more efficient than real computers) just to store the information in the human brain. The electrical power required to simulate its dynamics would be tectonic, and that's assuming perfect efficiency. Therefore, no computer that is composed of components which interact with the electromagnetic field and which operates at a temperature comparable to Earth temperatures will ever simulate a human mind to complete accuracy. It will always be an approximation.
• spangry 9y ago
  They really are terrifying. It's hard to say exactly why this is so, but I think it has something to do with how they can occur (some protein randomly misfolding) and that they aren't really "alive", in the same sense that viruses and bacteria are. You can kinda forgive viruses and bacteria: they're just looking to survive and multiply, just like us. In some cases they monopolise so much of their hosts' resources, or some really critical resource, that the host dies. But Prions, uhhhhh.... They're just kinda these unnatural things that go wrong and then kill you in pretty awful ways. Though not because they necessarily need to out-compete you for resources. Indeed, they don't seem to have any innate preference for existing or not existing, or thriving or not thriving. But they kill you anyway.... Also, just to scare you a little more, one of the more popular (perhaps dominant) theories about Alzheimer-family diseases is that they are initially caused by Prions, that migrate into your brain and start forming amaloid plaques, and then it's all down-hill from there. On the upside, I vaguely remember reading some research on a 'almost ready' drug, initially developed to lessen the negative side-effects of chemo-therapy, that might be effective at stopping the progression of these plaques as they spread throughout the brain. If it actually works, we might have a treatment of AD-family diseases on the market in a year or so (the chemo folks have already gone through the lengthy human safety testing part of the exercise). Not a cure, but at least it might halt or significantly slow progression of symptoms.