Speaker 1: Typically one day in middle age, the sufferer finds that
Speaker 1: he has begun to sweat. A look in the mirror
Speaker 1: will show that his pupils have shrunk to pinpricks, and
Speaker 1: he is holding his head in an odd, stiff way.
Speaker 1: Constipation is common. The women suddenly enter menopause and the
Speaker 1: men become impotent. The sufferer begins to have trouble sleeping
Speaker 1: and tries compensating with a nap in the afternoon, but
Speaker 1: to no avail. His blood pressure and pulse have become
Speaker 1: elevated and his body is an overdrive. Over the ensuing months,
Speaker 1: he tries desperately to sleep, sometimes closing his eyes, but
Speaker 1: never succeeding in falling into more than a light stupor.
Speaker 1: Their exhaustion is immense, beyond comprehension. Once the sufferer can
Speaker 1: no longer sleep, a downward progression ensues as he loses
Speaker 1: his ability to walk or balance. Perhaps most tragic, the
Speaker 1: ability to think remains intact. Sufferers know what is happening.
Speaker 1: At first, they can talk about it and even write
Speaker 1: down their thoughts. After a few more months, some lose
Speaker 1: this level of functioning once their bodies shut down. Only
Speaker 1: the desperate look in their eyes shows that they know
Speaker 1: what is going on, but others can talk and reason
Speaker 1: until the end. In the terminal phase, usually about fifteen
Speaker 1: months after the disease has begun, they fall into a
Speaker 1: state of exhaustion resembling a coma and die. Yeah.
Speaker 2: God, that's a bad one.
Speaker 1: Yeah. Do you want to hear what that is? Yeah,
Speaker 1: so that is a case of fatal familial insomnia, which
Speaker 1: is a preon disease. Welcome to preons everyone, And that
Speaker 1: is an excerpt from the book The Family Who Couldn't
Speaker 1: Sleep by DT Max, which is all about preons.
Speaker 2: Wow.
Speaker 1: Yes, hi, Hi, I'm Aaron.
Speaker 2: Welsh and I'm Erin alman Updyke.
Speaker 1: And this is this podcast will kill you.
Speaker 2: Yeah, today we're talking about preons.
Speaker 1: This is a big one. And we've said that before.
Speaker 2: We say that almost every time. Yeah, but it's always true,
Speaker 2: it is. Yeah, we haven't really covered any like baby diseases.
Speaker 1: Yet before we get into the nitty gritty of these
Speaker 1: many different diseases. What time is.
Speaker 2: It, it's quarantine any time.
Speaker 1: What are we drinking today?
Speaker 2: Well, this week we're drinking the chronically wasted because one
Speaker 2: of the prion diseases is chronic wasting disease.
Speaker 1: It has milk naturally, bourbon naturally, coffee naturally, no, just
Speaker 1: because those things taste good together, and then a little
Speaker 1: bit of cinnamon and nutmeg.
Speaker 2: And you just shake it all up and then strain
Speaker 2: it into a glass. And as always this season, we'll
Speaker 2: also be posting this recipe along with our place burrita,
Speaker 2: the non alcoholic version of our quarantines, on all of
Speaker 2: our social media accounts and our website, So check it
Speaker 2: out there.
Speaker 1: Excellent, Okay, preons. I have read so much about the history,
Speaker 1: about the whatever, but I really avoided the biology and
Speaker 1: understanding exactly what's going on, So please enlighten me.
Speaker 2: So, prions are a pathogen unlike any that we've ever
Speaker 2: looked at before, and unlike anything that we'll ever look
Speaker 2: at again, which is kind of thrilling.
Speaker 1: It really is.
Speaker 2: So every other infectious disease that we've discussed so far
Speaker 2: and that will ever discuss again has been either a
Speaker 2: virus or a bacterium or protozoan. In the future, will
Speaker 2: probably do a bunch of worms. But even at their
Speaker 2: most basic, all of these infectious diseases are some kind
Speaker 2: of organism which I'm putting in quotes because some people
Speaker 2: don't call viruses organisms, but at a bare minimum, even viruses,
Speaker 2: they've got either RNA or DNA and protein, and the
Speaker 2: genetic material is required because basically that's the only way
Speaker 2: that an organism can reproduce. The DNA or the RNA
Speaker 2: serve as templates to actually make the protein, and so
Speaker 2: then they also serve as the template that's replicated to
Speaker 2: make a new version of that organism. So for anything
Speaker 2: to be considered even close to a live and for
Speaker 2: sure to be able to reproduce, it has to have
Speaker 2: either DNA or RNA.
Speaker 1: Except for prerias.
Speaker 2: I'm excited. So preons are just protein, no nucleic acids,
Speaker 2: no genetic material, no DNA, no RNA, just protein. The
Speaker 2: word preon literally comes from protonaceous infectious particle. So creative.
Speaker 1: I it's it's so amazing to me because there's no
Speaker 1: biological incentive.
Speaker 2: Oh my god, you're still right.
Speaker 1: I don't get it. I don't get it. How why?
Speaker 2: Okay, I'm gonna try and answer it those two questions.
Speaker 2: As you can imagine, you are not the only person
Speaker 2: to feel like this, like what, how? Why? And I
Speaker 2: can't wait to hear the story, the story of how
Speaker 2: just mind blown scientists must have been when they finally
Speaker 2: were accepting that this was just a protein. It's oh,
Speaker 2: there's nothing like it. Okay, So here's how it goes.
Speaker 2: We're going to talk about proteins for a minute. Imagine
Speaker 2: that a protein is basically a rope. Okay. There are
Speaker 2: lots of different material that you can use to make rope,
Speaker 2: and there's lots of different lengths to which you can
Speaker 2: cut that rope. Just like protein in our body. We
Speaker 2: have lots and lots of different types of proteins. They're
Speaker 2: all made up of different amino acids, and they're all
Speaker 2: different lengths and sizes, and they all have different functions,
Speaker 2: and a large part of how that function is determined
Speaker 2: is how they're folded, or, if you're imagining our protein rope,
Speaker 2: how they're nottted. Okay, So think of how many different
Speaker 2: ways you can tie a knot. There are thousands, hundreds probably,
Speaker 2: And if you think of all of the different types
Speaker 2: of materials of rope, some of them are going to
Speaker 2: be better at holding certain knots than others. So proteins, yeah, right,
Speaker 2: this is a good analogy. I didn't come up with it,
Speaker 2: Brett did. So different materials of rope or different types
Speaker 2: of proteins are only going to be stable and therefore
Speaker 2: functional in certain conformations. Certain knots with me so far?
Speaker 2: Oh yeah, great. So in the case of preon diseases,
Speaker 2: there's this single protein, this single rope. It's called PRP,
Speaker 2: which just means get ready for the creativity here protein
Speaker 2: preon protein, no joke. So this protein is in your body.
Speaker 2: It's in your body, it's in my body, it's in
Speaker 2: everyone's who's listening's body. It's a cell membrane protein, which
Speaker 2: means it's found in the walls of your cells, in
Speaker 2: a whole bunch of our different cells, especially in our neurons.
Speaker 2: And we're not entirely clear what this protein does exactly.
Speaker 2: We don't know its exact function. We think it has
Speaker 2: to do with neuronal communication and the transport of stuff
Speaker 2: inside and outside the cell. That doesn't really matter right
Speaker 2: now for my purposes. The thing that matters is that
Speaker 2: this protein is normal most of the time, and when
Speaker 2: it's in its normal state, it's normal, not it's benign.
Speaker 2: It doesn't cause any disease. But for some reason, if
Speaker 2: it unknots itself and then renots itself in an abnormal way.
Speaker 2: It can begin to cause disease. And here's the scary part.
Speaker 2: When it interacts with other normal proteins in your body,
Speaker 2: it can cause them to change shape. And then those
Speaker 2: newly misfolded proteins interact with other normal proteins and cause
Speaker 2: them to change shape. And then do you see where
Speaker 2: I'm going with this.
Speaker 1: It's a domino effect of terrifying misfolded proteins.
Speaker 2: That's exactly what it is, and it's outrageous. It's similar
Speaker 2: in some ways to how a virus or a bacterium
Speaker 2: will get into your body and replicate and grow in number.
Speaker 2: But in this case, it's just a misfolded version of
Speaker 2: a protein that's already in your body, and in its
Speaker 2: normal form, it's not a big deal. But if it
Speaker 2: comes into contact with a misfolded version, it becomes misfolded
Speaker 2: and that's how it replicates. And exactly how this happens
Speaker 2: to begin with, we don't know. Down Damn, I'm just struggling,
Speaker 2: struggling for words right now.
Speaker 1: I'm struggling for words because it is. It sounds like
Speaker 1: it's from a sci fi book.
Speaker 2: Yeah, it totally does.
Speaker 1: And it's it's just very it's it's outside of this
Speaker 1: this paradigm of infection or disease or proteins that we have,
Speaker 1: all that we have been taught throughout school, throughout life. Whatever.
Speaker 2: Yeah, we kind of mentioned this at the beginning, but
Speaker 2: there are a lot of different names for preon diseases.
Speaker 2: But the thing that I did not realize until starting
Speaker 2: to research for this episode is that all of these
Speaker 2: different diseases that you've probably heard of are all caused
Speaker 2: by misfolding of the same protein, the same one, that
Speaker 2: PRP protein. It's not tons of different proteins in a
Speaker 2: cow versus a sheep versus a human, It's the same one.
Speaker 2: How how how great question. Honestly, I did not know
Speaker 2: that at all. Now. The thing is, each of these misfoldings,
Speaker 2: these different knots in the rope, they're slightly different versions,
Speaker 2: so they kind of refer to them as different strains,
Speaker 2: the way that we would call different strains of ebola virus.
Speaker 2: It's the same virus, but there are multiple strains. It's
Speaker 2: similar in this except that, again, it's just a protein,
Speaker 2: but they're all misfolded in slightly different ways. So these
Speaker 2: different variants of the misfolding of the preon protein. They
Speaker 2: cause different diseases that were named by and classified by
Speaker 2: their incubation period, which varies really widely, and the actual neuropathology.
Speaker 2: So the way that they affect your brain and the
Speaker 2: symptoms that they cause are actually a little different in
Speaker 2: each version of preon disease, even though it's the same
Speaker 2: misfolded protein. So what I'm going to do is go
Speaker 2: through the different human diseases, and I am going to
Speaker 2: focus on human diseases, but we can talk about some
Speaker 2: of the other mammal diseases too, because they're also really interesting.
Speaker 1: Don't worry, I'll get there.
Speaker 2: Oh good, So we'll go through the different human ones,
Speaker 2: but first I just want to talk to you about
Speaker 2: what they all have in common. So the other name
Speaker 2: for preon diseases is transmissible sponge ofform encephalopathy, which janus
Speaker 2: so what does that mean? It means this transmissible just
Speaker 2: tells us that this is an infectious disease, meaning it
Speaker 2: can be passed from one person to another, or in
Speaker 2: some cases, from one mammal to another. Incephalopathy just means
Speaker 2: it affects your brain and sponge ofform tells us how
Speaker 2: it affects your brain. It actually causes spongeiform changes, so
Speaker 2: your brain becomes wholly like a sponge. And importantly, this
Speaker 2: these holes that happen, it happens without any inflammation. So
Speaker 2: inflammation is a response of our body. It's a natural
Speaker 2: response to either tissue damage or the presence of some
Speaker 2: kind of non self like bacteria or viruses. But in
Speaker 2: this case, with preons, your body doesn't react to the
Speaker 2: preon proteins or to the damage they cause with normal
Speaker 2: inflammatory responses. So you have neurons and other brain cells
Speaker 2: actively dying, leaving gaping holes empty space in their place,
Speaker 2: with no white blood cell invasion, no activation of the
Speaker 2: inflammatory cascade or anything like that. So that's what they
Speaker 2: have in common. Let's talk briefly about the different variance.
Speaker 1: Of this disease.
Speaker 2: Okay, so the first one, since we touched on it
Speaker 2: briefly in our first hand account, is fatal familial insomnia. Now,
Speaker 2: this is a weird one. It's super super super rare,
Speaker 2: like very rare, but it causes insomnia, that's one of
Speaker 2: the major symptoms. It also causes speech and physical coordination
Speaker 2: problems and dementia. This one is not necessarily infectious, but
Speaker 2: it is familial, so it's actually transmitted genetically. And it's
Speaker 2: autismal dominant, which means that if one of your parents
Speaker 2: has it, you have a fifty percent chance of getting
Speaker 2: it as.
Speaker 1: Well, like Huntington's.
Speaker 2: Exactly, just like Huntington's. So it's a weird one. Okay,
Speaker 2: the next one, we're just gonna blow through these. The
Speaker 2: next one is called GSS Gershmann Straussler Schineker syndrome. Was
Speaker 2: that okay, I don't know. I don't know either. I
Speaker 2: don't speak German. This is another weird one. It's also familial,
Speaker 2: so just like fatal familial insomnia, it doesn't cause insomnia though. Instead,
Speaker 2: what happened is you first start with dysarthria, which means
Speaker 2: difficulty speaking, so you might not be able to talk.
Speaker 2: And then you'll get what's called a taxia, which means
Speaker 2: you're you can't coordinate your body movements, so you might
Speaker 2: have tremors, you might have an unsteady, wobbling gait. And
Speaker 2: then you'll progress to dementia, memory loss, visual disturbances, and
Speaker 2: eventually death.
Speaker 1: That sounds like it's going to be the common thread,
Speaker 1: and I know that it is, but.
Speaker 2: It is the other thing that I didn't mention that
Speaker 2: ties all of these together is that they are one fatal.
Speaker 1: Cool. Yeah, so that's our second this season.
Speaker 2: Yeah. Well, I mean Raby's was like almost one hundred percent.
Speaker 2: At least you can treat it. If you can't, Yeah,
Speaker 2: we've got to have one.
Speaker 1: Yeah.
Speaker 2: Now, let's get into the diseases. You've probably heard more
Speaker 2: about kuru Have you heard of it?
Speaker 1: Oh?
Speaker 2: Well, I know you have listeners. Have you tell us?
Speaker 2: Kourou is a disease that is transmissible and it's believed
Speaker 2: to have been transmitted to people from the consumption of
Speaker 2: deceased family members during burial rituals, which I'm guessing you're
Speaker 2: going to talk a lot more about, Aaron. Oh yeah,
Speaker 2: I avoided the history of this. What I do know
Speaker 2: is that this was one of one of the early
Speaker 2: preon diseases that people sort of found out about, right, Yeah,
Speaker 2: And one of the first ways that we figured out
Speaker 2: that this was actually a transmissible disease and how people
Speaker 2: actually got it from eating meat that had been contaminated
Speaker 2: with preon proteins. So for this one, the symptoms start
Speaker 2: often with body tremors, so all over body tremors and
Speaker 2: then bursts of laughter, which I find so interesting and
Speaker 2: scary because what was it was described as people would
Speaker 2: become very depressed, which is understandable, but then they would
Speaker 2: have these bursts of uncontrollable laughter that they just couldn't
Speaker 2: couldn't stop.
Speaker 1: Yeah.
Speaker 2: Yeah. Then you would also get that same ataxia, so
Speaker 2: tremors combined with a wobbly gait and just not being
Speaker 2: able to coordinate your body movements. And then in the
Speaker 2: later stages you can actually get ulcerations, so open wounds,
Speaker 2: like on various places of your body that lead to
Speaker 2: secondary infection. Oh yeah, and so actually one of the
Speaker 2: major causes of death in Kuru is pneumonia or another
Speaker 2: secondary infection, not necessarily the preon proteins itself.
Speaker 1: Interesting. I didn't know that.
Speaker 2: Yeah huh. And then we have the most famous or
Speaker 2: should I say actually the two most famous probably for
Speaker 2: of preon disease in humans krutz feld yakup disease also
Speaker 2: variant kruzfeld yacup disease. There are two there's actually more
Speaker 2: than two forms, but these two forms are very distinct.
Speaker 2: So let's talk about the classic. First, classic CJD is sporadic.
Speaker 2: This is not what you get from eating cow meat
Speaker 2: that's been contaminated. This is not mad cow. So krutzveeld
Speaker 2: yakup disease is a preon disease that we don't know
Speaker 2: why or how it happens. We think maybe it's just
Speaker 2: age related random mutations. Aw who knows. You can get them.
Speaker 2: You can get them from things like corneal transplants. There
Speaker 2: have been several documented cases of people who received cornea
Speaker 2: transplants from someone who had documentedly who had died from
Speaker 2: kruzfeld yakup disease, who then got krus folt yacka disease
Speaker 2: and died from the cornea transplant, and also dura mater transplants.
Speaker 2: Dura mater is the outer layer of your brain mi ninja,
Speaker 2: so like the tissue that covers your brain. Essentially okay,
Speaker 2: Sometimes you might do a graft of that from one
Speaker 2: person to another in the case of some extreme trauma,
Speaker 2: and if that person died from krus folt yakub, then
Speaker 2: you will also. So this type of CJD is characterized
Speaker 2: by a rapidly progressive dementia and memory loss. Those are
Speaker 2: the first two things that tend to happen, and then
Speaker 2: you'll get personality changes hallucinations.
Speaker 1: So when you say rapidly progressive, what's what's the timeframe
Speaker 1: we're looking at.
Speaker 2: So most of the time that was something I didn't
Speaker 2: mention about all of these diseases, but they all have
Speaker 2: very different durations of illness. So fatal familial insomnia, the
Speaker 2: average age at death is fifty and the average duration
Speaker 2: of illness, so from the time you start showing symptoms
Speaker 2: until you die, is eighteen months for f FI. Okay,
Speaker 2: for Gershmann, Straussler, Shinecker, GSS, the median age at death
Speaker 2: it's really variable because it's such a small population, but
Speaker 2: the duration of illness is anywhere from three months to
Speaker 2: thirteen years, with an average of five years.
Speaker 1: So too much.
Speaker 2: God, Yeah, it's a very long progressing disease. Kuru, the
Speaker 2: duration of illness is only about twelve months, but they
Speaker 2: think that the incubation period could be as long as
Speaker 2: fifty years. That's the time from when you first get
Speaker 2: exposed to prion's until they build up in enough number
Speaker 2: in your brain to actually start causing disease. But with
Speaker 2: Krutzfelt Yakub classic CJD. The median age at death is sixty,
Speaker 2: but the duration of illness is only four to six months,
Speaker 2: So once you start showing symptoms, you're probably dead within
Speaker 2: five months.
Speaker 1: Holy cow.
Speaker 2: The longest that's been documented that somebody has survived after
Speaker 2: starting to show symptoms is about two years with classic CJD. Wow. Yeah,
Speaker 2: so very rapidly progressive. You'll also end up with hallucinations,
Speaker 2: mild clonus, which means muscle spasms, and then late in
Speaker 2: the disease you can have things like a taxia again,
Speaker 2: that wobbly gait, So we're seeing some overlap with symptoms
Speaker 2: speech impairment again. Here the cause of death tends to
Speaker 2: be pneumonia, which I find very interesting, especially that it
Speaker 2: can happen that rapidly.
Speaker 1: Well, and also I don't understand the mechanism behind, Like
Speaker 1: this is a neurological disorder, Tell me about it.
Speaker 2: Yeah, so, how how well if you're if it's affecting
Speaker 2: the parts of your brain that cause you to breathe normally,
Speaker 2: and if you're not breathing normally, or if you're not
Speaker 2: coughing up normally, or if it's even just affecting like
Speaker 2: all of the functions of your organs, right, everything is
Speaker 2: controlled by your brain. It's maybe impairing more of our
Speaker 2: unconscious functions than it is our conscious functions. Okay, and
Speaker 2: then the last human preon disease that we need to
Speaker 2: talk about is variant Krutzfeld yakup. This is the one
Speaker 2: associated with mad cow. So this is the one which
Speaker 2: I'm sure you'll talk about the outbreak that started it all,
Speaker 2: don't you know it where a bunch of people became infected,
Speaker 2: presumably after eating contaminated cow meat beef that had those
Speaker 2: cows had died from mad cow, also known as bovine
Speaker 2: sponge offm encephalopathy or BSE. So that is the form
Speaker 2: of preon disease that happens in cows, and if you
Speaker 2: eat a cow that's died from that, you will get
Speaker 2: v CJD, very different from classic CJD. The median age
Speaker 2: at death is thirty or under, so these were young
Speaker 2: people who are affected, and the median duration of illness
Speaker 2: is thirteen to fourteen months. So it's a longer duration
Speaker 2: of disease.
Speaker 1: Hm hm, possibly because it came from.
Speaker 2: Cal Yeah, yeah, so it it presents in a way
Speaker 2: that's much more similar to KURU, and that's part of
Speaker 2: the reason that they were able to make that link
Speaker 2: between vCJD and KURU both being transmitted by consuming preon
Speaker 2: infected meats, because they do present very similarly. With v CJD.
Speaker 2: It's hard acronym to say, you present not so much
Speaker 2: with dementia and memory loss like with classic Chris felt Yaka,
Speaker 2: but with other psychiatric and behavioral symptoms, and also very
Speaker 2: classically with very painful what's called diastesias, which means nerve
Speaker 2: pain and like your nerves are firing in ways that
Speaker 2: is really painful and not under your control, so maybe
Speaker 2: you'll have hands cramping and things like that. Yeah, you
Speaker 2: don't get as much of the ataxias and things with
Speaker 2: v CJD as you do with some of the other diseases,
Speaker 2: but you do die just like with all the others.
Speaker 2: And one of the scary things about vCJD is that
Speaker 2: it is believed that it can also be transmitted by
Speaker 2: blood products. So that means like blood products, what do
Speaker 2: you that's another way to say that sirum serum. Yeah,
Speaker 2: if you give blood transfusion, That's what I'm trying to say.
Speaker 2: Whereas there isn't really evidence that other forms of this
Speaker 2: disease can be transmitted in that way. Oh yeah. So,
Speaker 2: while you can get CJD from corneo graphs or dural
Speaker 2: graphs or things where you're actually coming in contact with
Speaker 2: neuronal surfaces, there is an evidence that you can get
Speaker 2: it from blood products. But with v CJD you can
Speaker 2: potentially transmit that because you do find preon proteins in
Speaker 2: the peripheral blood, not just in the nervous system.
Speaker 1: That's very strange, yes, huh yeah.
Speaker 2: And the other thing that's similar between KURU and vCJD
Speaker 2: is that you get more deposition of the actual preon
Speaker 2: proteins into plaques, which means that these misfold proteins aggregate,
Speaker 2: clump together, and deposit into your brain in a similar
Speaker 2: way that you get aggregates in something like Alzheimer's. And
Speaker 2: it's also we have no idea how long the incubation
Speaker 2: period is for these two diseases. These are the most
Speaker 2: sort of transmissible of the transmissible sponge ofform and cephalopathies
Speaker 2: in humans, and we don't know how long ago some
Speaker 2: people or infected.
Speaker 1: It could be.
Speaker 2: In some cases, it seems to be a matter of months.
Speaker 2: In other cases it could be twenty thirty, forty years.
Speaker 2: So a lot of people think that they are going
Speaker 2: to be more and more cases of vCJD down the
Speaker 2: line from people who were exposed back in the nineties.
Speaker 1: That's alarming.
Speaker 2: It's alarming, and probably the most alarming thing about preons
Speaker 2: is that we can't do anything about them. So there's
Speaker 2: no treatment, is it?
Speaker 1: Because it's a protein that's in your body, and so
Speaker 1: by attacking that protein, you're liable to attack other proteins
Speaker 1: that you actually need and function.
Speaker 2: It's part of it. Yeah, But I mean cancer cells
Speaker 2: are also your body cells, but we have ways that
Speaker 2: we can kill those. The reason it's so hard to
Speaker 2: deal with preons is because most of the drugs that
Speaker 2: we use to target diseases, infectious diseases and things like
Speaker 2: cancers target DNA A target reproduction in the way that
Speaker 2: we know that reproduction happens. We don't know why this
Speaker 2: protein becomes misfolded, and we don't know how it causes
Speaker 2: other proteins to become misfolded, so we don't know how
Speaker 2: to target it. And because we don't know exactly what
Speaker 2: the PRP protein does normally, it makes it even harder
Speaker 2: to actually find ways to try and stop it from
Speaker 2: misfolding or you know, make it go back to its
Speaker 2: normal confirmation, Yeah, is there.
Speaker 1: Is there at least promising work on stuff like how
Speaker 1: it's analogous to Alzheimer's or is that telling us anything
Speaker 1: more about these other neurodegenerative diseases.
Speaker 2: It's a good question. We'll talk a little bit more
Speaker 2: about it in this sort of current events section. There's
Speaker 2: definitely a lot of parallels, especially in terms of understanding
Speaker 2: how proteins fold and why they misfold in certain ways.
Speaker 2: These don't tend to be misfolded, at least from what
Speaker 2: I read, they don't tend to be misfolded in the
Speaker 2: same ways that Alzheimer's proteins are misfolded. So it's not
Speaker 2: the same conformations that cause Alzheimer's plaques, if that makes sense.
Speaker 2: But yeah, we don't have we don't have a lot currently,
Speaker 2: and they're also just really hard to get rid of.
Speaker 2: Like if your meat becomes contaminated, there's not much that
Speaker 2: you can really do about it because proteins don't degrade
Speaker 2: as easily as DNA or RNA. So the ways that
Speaker 2: we normally use to sterilize. Things don't always work on preons.
Speaker 2: They're not impossible to kill, though people who are like
Speaker 2: you can't have a kill a preon, like that's not true.
Speaker 2: It just takes a lot higher heat. It takes things
Speaker 2: that dnature proteins rather things that rather than things that
Speaker 2: dnature DNA and RNA, So it's different techniques. It is possible,
Speaker 2: though it's not impossible.
Speaker 1: Right.
Speaker 2: Yeah, how's that?
Speaker 1: That was pretty once, very scary introduction to Yeah, we
Speaker 1: didn't even.
Speaker 2: Talk about scrapy or BSSE or chronic wasting disease, which
Speaker 2: I think is actually the scariest one. So Aarin, tell
Speaker 2: me what is up with these preons?
Speaker 1: How did they get here?
Speaker 2: And why should I be so afraid of them?
Speaker 1: I think that last question you're going to have to answer. Actually,
Speaker 1: I think that last question you just answered.
Speaker 2: Oh okay, well, then just tell me how they got
Speaker 2: here and what's going on with them?
Speaker 1: Okay, happy to do that. I'm mourning you.
Speaker 2: Now.
Speaker 1: The history of prions is a huge one, in part
Speaker 1: because it's not really just one disease like you just
Speaker 1: talked about. Each prion disease has its own detective story,
Speaker 1: and each contributes to the story of prions as a whole,
Speaker 1: and that story is going to take us all over
Speaker 1: the world, from the pastures of Spain to the highlands
Speaker 1: of Papua New Guinea, from the grocery stores of Britain
Speaker 1: to the forests of North America. And we're going to
Speaker 1: meet some rather interesting people along the way who will
Speaker 1: show us that changing people's minds can be exceptionally hard,
Speaker 1: especially when ego and glory seem to be driving you
Speaker 1: more than a quest for truth. Let's get started. Even
Speaker 1: though the word prion was only invented a few decades ago,
Speaker 1: the history of prion disease is which is back much farther,
Speaker 1: probably hundreds of thousands of years. But before I get
Speaker 1: to that, let's try to track when preon diseases first
Speaker 1: became known to modern humans. There are many early references
Speaker 1: to a pre on like condition that have been put
Speaker 1: forth as evidence for the disease being present at a
Speaker 1: certain time or place. Hippocrates may have mentioned it. There's
Speaker 1: strong support that Shakespeare refers to it in Macbeth. Yeah,
Speaker 1: I didn't read it, but a lot of things are like,
Speaker 1: oh yeah in Macbeth, blah blah blah, I should probably
Speaker 1: find that line, and it was probably around during those times.
Speaker 1: But let's get down to when it first started making
Speaker 1: big waves. Between the eighteenth and nineteenth centuries in Europe,
Speaker 1: human population was on the rise, and that meant figuring
Speaker 1: out ways to make more food, to make more clothing, housing,
Speaker 1: et cetera, to support the growing populace. And one way
Speaker 1: that people maximized efficiency and productivity was through selective breeding,
Speaker 1: which wouldn't actually go by that name for almost one
Speaker 1: hundred years when Darwin would discuss it in his books,
Speaker 1: but the concept of breeding plants or animals to select
Speaker 1: for certain desired traits was known, and one of the
Speaker 1: biggest developers of selective breeding was a dude named Robert Bakewell,
Speaker 1: who applied it to sheep and in doing so changed
Speaker 1: the course of history.
Speaker 2: Always the bake wells with the sheep.
Speaker 1: What is the fastest way to get the traits that
Speaker 1: you want in a sheep?
Speaker 2: I don't have the slightest idea.
Speaker 1: Okay, well, you would breed the sheep who already look
Speaker 1: the closest to your ideal image.
Speaker 2: Okay, that makes sense.
Speaker 1: Bakewell started doing what was known as in and in breeding.
Speaker 1: Uh uh yeah, which means mating parents with offspring offspring
Speaker 1: with each other. Really Game of Thrones type.
Speaker 2: I was gonna say, just like you know England, but.
Speaker 1: To day.
Speaker 2: You know.
Speaker 1: This, This practice wasn't really done because it was common
Speaker 1: knowledge among farmers that inbred animals tended to have more
Speaker 1: hereditary defects. But the results that Bakewell had gotten in
Speaker 1: a really short amount of time were too impressive to ignore,
Speaker 1: and soon enough Bakewell was studding out his best rams,
Speaker 1: and farmers everywhere started their own in and in breeding practices.
Speaker 1: In those years, the relatedness of those sheep sharply increased,
Speaker 1: as you might expect, and things were about to get
Speaker 1: a whole lot more related. English sheep weren't known for
Speaker 1: their fleece, unlike the Spanish Merino breed.
Speaker 2: Your favorite sheep.
Speaker 1: I believe Marino wool is one of my absolute favorite things.
Speaker 2: On Yeah I know.
Speaker 1: So anyway, Yeah, so a guy named Banks decided to
Speaker 1: turn a profit by shipping a handful of Spanish Merino
Speaker 1: sheep to farmers all over England where they were bred
Speaker 1: in and inn even more so they could get that
Speaker 1: nice fleece. By the end of the seventeen hundreds, a.
Speaker 1: Most of England's sheep were intensively in bread and b
Speaker 1: The inbreeding was not in pockets, but rather all over.
Speaker 1: It was the only way you could maintain sheep.
Speaker 2: Uh.
Speaker 1: Then a few sheep started acting strange. They seemed to
Speaker 1: have an itch. They could not scratch enough.
Speaker 3: OHI.
Speaker 1: They would rub their heads and their rumps on posts,
Speaker 1: on trees, on rocks, on fences, on anything to try
Speaker 1: to get some kind of relief. But this was a
Speaker 1: one direction disease. The sheep never recovered from their itch,
Speaker 1: and later stages staggered around until they suddenly dropped dead.
Speaker 1: Soon this phenomenon was all over England and Scotland, earning
Speaker 1: various names along the way, such as Rubbers and Yuki Pine,
Speaker 1: the Frenzies, the giddies, Scratchy Shrewcroft, turn sick, the disease,
Speaker 1: the disease no like getting dizzy, Oh the dizzy.
Speaker 2: It's like, I'm sorry, Preon is now officially the least
Speaker 2: creative group of people. We're just gonna sell this one
Speaker 2: of the disease.
Speaker 1: I kind of like it. It's like the band, Yeah,
Speaker 1: just classic, the shaking, the mad Staggers, which might be
Speaker 1: my favorite, the goggles, and finally the one that stuck
Speaker 1: with it. Everywhere sheep were dropping by the dozens hundreds,
Speaker 1: and no one knew what was causing it. Hypotheses, of
Speaker 1: course abounded. It was the air, It was a maggot,
Speaker 1: It was too much sex, it was not enough sex.
Speaker 1: It turned out that it had previously been described by
Speaker 1: Spanish shepherds decades before, but this outbreak probably wasn't caused
Speaker 1: by the import of those sheep, just a matter of
Speaker 1: high sheep density and a lack of scrapy resistance in
Speaker 1: the inbread sheep. No real progress would be made for
Speaker 1: years on what the actual cause of scrapey was, but
Speaker 1: solving that mystery lost a little bit of its urgency
Speaker 1: as scrapy resistant sheep breeds began to take over. Wow,
Speaker 1: let's say goodbye for now to our beautiful Merino sheep
Speaker 1: in England and traveled to Papua New Guinea in the
Speaker 1: nineteen fifties, which was at the time partially under Australian rule.
Speaker 1: The Australians in charge didn't really know a lot about
Speaker 1: the islands culture's ecology, et cetera, but they were looking
Speaker 1: to change that. In particularly, they wanted to know who
Speaker 1: was living in the dense rainforests of the Highlands to
Speaker 1: make contact. I'm saying that in air quotes rather than conquer.
Speaker 2: Yeah.
Speaker 1: Anyway. One of the groups that was contacted was called
Speaker 1: the Fora. Right away, those making the contact noticed a
Speaker 1: couple of strange things. For one, there weren't many women around,
Speaker 1: but there were a lot of unmarried men, and after
Speaker 1: spending a bit more time with them, they noticed a
Speaker 1: mysterious illness that was circulating, resulting in a high number
Speaker 1: of deaths. Those afflicted, mostly women, would shiver and jerk
Speaker 1: uncontrollably for weeks, occasionally hit as you mentioned by this
Speaker 1: involuntary hysterical laughter, and then this would continue until they died.
Speaker 1: The Fora called it shaking or kuru, and it was
Speaker 1: devastating Phila. Seeing this, the Australians decided to send for
Speaker 1: a doctor to investigate what exactly was going on. The doctor,
Speaker 1: a man named Victor Ziegas, interviewed sufferers and took many
Speaker 1: samples to send off for analysis, but no one could
Speaker 1: find anything in the blood or serum or even brain samples.
Speaker 1: He sent no bacterium, no virus, no fungus, no worm,
Speaker 1: nothing thing enter Carlton Gadgetsek. I want to pause here
Speaker 1: and warn anyone who is listening that I'm going to
Speaker 1: talk a bit about Gadgessec's criminal history, which includes his
Speaker 1: conviction as a child molester. So if you don't want
Speaker 1: to hear about that, fast forward about two and a
Speaker 1: half minutes. Let me give you a little bit of
Speaker 1: background on this piece of work. Gadgasek was a convicted
Speaker 1: child molester and self proclaimed pedophile. He was also a
Speaker 1: Nobel Prize winning pediatrician.
Speaker 2: I'm sorry what?
Speaker 1: Oh yeah, yep. Medically, Gadgisik was fascinated by rare, incurable diseases. Personally,
Speaker 1: he was interested in so called non Western sexuality, particularly
Speaker 1: as it related to children.
Speaker 2: Ew I hate this already.
Speaker 1: Yeah, he's a despicable human being. He was not well
Speaker 1: liked by many of his colleagues, or liked at all,
Speaker 1: and some would refer to him as inhuman and just
Speaker 1: short of a sociopath. Along the path of his research
Speaker 1: were scattered the remains of former collaborations and friendships. At
Speaker 1: the same time, Gadgisik was widely admired by his academic
Speaker 1: peers for his drive, ambition, and intelligence. And many of
Speaker 1: these peers would later beg leniency from the judge for
Speaker 1: Gadgetiic sentencing for child molestation. Quote, Carlton was a complicated man,
Speaker 1: his peers would say, or something along the lines of of, no,
Speaker 1: he wasn't perfect, but he was a genius, which seemed
Speaker 1: to excuse his behavior as a child molester as the
Speaker 1: cost of conducting important medical research.
Speaker 2: Oh my god.
Speaker 1: Or they would take a stance of cultural relativism. If
Speaker 1: pedophilia was practiced in a certain culture, then it was okay.
Speaker 1: If Gadgaseek sought out those cultures as his hunting ground. Ew, yeah,
Speaker 1: they would they that was part of his defense. Oh.
Speaker 1: The reason that I'm spending so much time talking about
Speaker 1: who Gadgetick was is because I am extremely frustrated with
Speaker 1: the fact that his molestations, his crimes, are so often
Speaker 1: relegated to a footnote when discussing him or his research. Yeah,
Speaker 1: it's so frustrating to me when I read an entire
Speaker 1: book about preons, The Family who Couldn't Sleep discusses it
Speaker 1: in depth, but Stanley Prisoner's book very briefly mentions this,
Speaker 1: and it is so appalling because.
Speaker 2: Then it just like it almost excuses it, like we
Speaker 2: can just ignore that piece of history exactly.
Speaker 1: So now that you know who Gadgisick was, let's get
Speaker 1: back to Papua and New Guinea. In the nineteen fifties,
Speaker 1: Gadgisick was always on the hunt for new diseases and new,
Speaker 1: in his words, uncivilized places to explore. When he was
Speaker 1: in Australia looking for his next adventure, he learned about
Speaker 1: this mysterious illness that was devastating the foray this could
Speaker 1: be the medical find of the century, and he wanted
Speaker 1: to be a part of it, not just a part
Speaker 1: of it, the whole of it. He shouldered his way
Speaker 1: into the investigation and took it over, irritating many people
Speaker 1: along the way who started to wonder if he had
Speaker 1: a medical degree at all.
Speaker 2: Wow.
Speaker 1: Yeah. Once in Papua New Guinea, he took samples and
Speaker 1: more samples and tracked cases as the numbers climbed every month.
Speaker 1: In June of nineteen forty seven, he had recorded over
Speaker 1: two hundred deaths, and these deaths followed a strange pasath.
Speaker 1: It appeared that for every one male struck by KUU,
Speaker 1: up to fourteen females were sick with the disease, and
Speaker 1: still the origin of this disease, or even its pathology,
Speaker 1: had yet to be figured out. But slowly the pieces
Speaker 1: were falling into place. First, a researcher working in one
Speaker 1: of the labs to which Gadgasik had sent victims' brains
Speaker 1: noticed strange deformities in some nerve cells, and these deformities
Speaker 1: reminded him of a disease he had just seen in
Speaker 1: a textbook, Krutzfeldt Yakub disease.
Speaker 2: Oh yeah.
Speaker 1: Anxious to make similar headway on the source of the disease,
Speaker 1: gadgasek supervisor told him he wanted to assemble a team
Speaker 1: of anthropologists, epidemiologist, physicians, et cetera. But Gadgasik refused. Couldn't
Speaker 1: they understand he was the team?
Speaker 2: Oh my god.
Speaker 1: Yeah, But he wouldn't end up actually solving that riddle,
Speaker 1: even though he would later claim to have known about
Speaker 1: the disease's origin all along.
Speaker 2: Oh cool, Yeah, No, I knew about it. I just
Speaker 2: didn't tell you guys.
Speaker 1: Yeah, he said it was too obvious to publish.
Speaker 2: Oh my god. Yeah, I don't know that I've ever
Speaker 2: hated a person that we've talked about on this podcast more.
Speaker 1: I despise him. No, the origin of the disease would
Speaker 1: actually be uncovered by anthropologist Shirley Lindenbaum and medical researcher
Speaker 1: Michael Alpers.
Speaker 2: Yeah, Sureley and Michael too.
Speaker 1: They did the careful, methodical work that Gadgasek never had
Speaker 1: the time for, like drawing family trees and tracing relationships,
Speaker 1: and more importantly, they listened to the Foray. Since the
Speaker 1: disease was relatively new to the Foray, dating back only
Speaker 1: forty or fifty years, whatever had caused it was probably
Speaker 1: new as well. And while numerous people had noted their
Speaker 1: observations of cannibalism practiced by the Fora, what they didn't
Speaker 1: know was what these two researchers would learn by listening.
Speaker 1: Cannibalism had only been adopted by the Forae about fifty
Speaker 1: years prior Aha Yeah, timelines line enough. This wasn't revenge
Speaker 1: cannibalism eating their enemies. The Fora used cannibalism to honor
Speaker 1: and mourn their dead loved ones, and this practice seemed
Speaker 1: to be at the root of the Kuru epidemic. It
Speaker 1: explained the recent emergence of the disease and the skewed
Speaker 1: sex ratio, and when the Forae stopped practicing cannibalism at
Speaker 1: the insistence of some missionaries, the disease started its decline.
Speaker 1: This was a controversial explanation for many reasons. But if
Speaker 1: this was an infectious disease, it was unlike any that
Speaker 1: had been described. The years long, decades long incubation period.
Speaker 1: The absence of any detectable virus or bacterium, it was bizarre.
Speaker 1: Around nineteen sixty, Gadgasek returned to the US to work
Speaker 1: on Kuru in a lab setting when he was contacted
Speaker 1: by a Scrapie researcher named William Hadlow, who had seen
Speaker 1: an exhibit on Kuru in a museum that included pictures
Speaker 1: of brain tissue showing the damage. Oh Hadlow thought that
Speaker 1: Kuru looked strikingly similar to Scrapie, so he published this
Speaker 1: observation and also wrote to Gadgetsek directly earlier. I left
Speaker 1: off with Scrapie in the early eighteen hundreds, but the
Speaker 1: disease hadn't disappeared. Occasional outbreak still occurred in sheep all
Speaker 1: over Europe, and it showed up an American sheep in
Speaker 1: nineteen forty seven, so it was still getting a lot
Speaker 1: of research attention. The biggest development with Scrapye, though, came
Speaker 1: about when it was shown to be transmissible after thousands
Speaker 1: of sheep developed the disease after being given a vaccine
Speaker 1: for looping ill. For what looping ill? It's a tick
Speaker 1: or illness?
Speaker 2: What a ridiculous name.
Speaker 1: We'll cover it, We'll cover someday. Good. The vaccine had
Speaker 1: been prepared from sheep brains that had been treated with formulin,
Speaker 1: which should kill pretty much all living things. It sure should,
Speaker 1: but not Preon's okay.
Speaker 2: Also, people don't get scared of vaccines because of this,
Speaker 2: because we don't make vaccines from brains for humans. Okay, now,
Speaker 2: we don't relax.
Speaker 1: This also showed that there could be a very long
Speaker 1: period from exposure to development of symptoms, just like Kuru.
Speaker 1: The note from Hadlow gave Gadgetsek the idea to see
Speaker 1: whether Kuru could also be transmitted. He set about acquiring chimpanzees,
Speaker 1: other primates, mice, hamsters, basically whatever he could get his
Speaker 1: hands on to inject with tissue from Kuru victims. He
Speaker 1: left someone else in charge of doing the actual work
Speaker 1: and went to go explore new places. Within twenty one months,
Speaker 1: which is an eternity. In the lab, a chimpanzee named
Speaker 1: Georgette started showing signs of the disease. Wow.
Speaker 2: I never thought about how difficult it would be to
Speaker 2: do research on Preon's in the lab because of how
Speaker 2: long the incubation period is.
Speaker 1: It's really difficult, and I think very emotionally taxing.
Speaker 3: Yeah.
Speaker 2: Also, it's a gamble, right, yeah, ma'am.
Speaker 1: One by one the rest of the chimps developed what
Speaker 1: looked like Kuru and died. Oh poor babies, I know. Georgette.
Speaker 1: The damage to their brains looked incredibly similar to that
Speaker 1: of Kuru scrapy and kruz felt yakub sufferers and Gayjsek
Speaker 1: ordered more injections, not just with Kuru, but with tissue
Speaker 1: from other neurodegenerative diseases as well. These experiments show that Kuru, scrapy,
Speaker 1: and kruzfeld yaka could all be transmitted. He published his
Speaker 1: results and hypothesized that these three diseases were all caused
Speaker 1: by the same thing, a slow vibe, a vague explanation
Speaker 1: that he didn't really expand on. For this and for
Speaker 1: his description of Kuru, he would be awarded the Nobel
Speaker 1: Prize in nineteen seventy six. Jeez, yeah, a decision that
Speaker 1: baffled many, including me.
Speaker 2: Can they take it away?
Speaker 1: Like?
Speaker 2: Does that ever get taken away?
Speaker 1: That's a great question. I don't know. We should look
Speaker 1: into that.
Speaker 2: We should look into it.
Speaker 1: Meanwhile, plenty of other researchers were hard at work on
Speaker 1: these diseases, such as Tikva Alper, who demonstrated that even
Speaker 1: radiation could not destroy the scrapy particle. WHA. I also
Speaker 1: have to say she sounds amazing. So she refused to
Speaker 1: accept a royal sixtieth wedding anniversary greeting from the Queen
Speaker 1: because it was addressed to her and her husband under
Speaker 1: his name, even though she didn't take his last name.
Speaker 2: Oh my god, she is. I I feel so strongly
Speaker 2: about Whenever we get a letter that's addressed to mister
Speaker 2: and missus Brett Updike, I throw it at him and
Speaker 2: I'm like, this is for you, which like it's not
Speaker 2: his fault, but oh my god, I feel that so strongly.
Speaker 1: She's awesome. Another big discovery was that there seemed to
Speaker 1: be multiple strains of scrapie, judging by the different patterns
Speaker 1: of disease. But still, what was the agent? Where was
Speaker 1: the virus? How could it survive being irradiated, desiccated, cooked.
Speaker 1: Researchers could isolate the protein coat of this mysterious virus
Speaker 1: that supposedly existed, but they couldn't detect any nucleic acids.
Speaker 1: I'm getting so excited.
Speaker 2: I'm sorry, I'm getting so excited.
Speaker 1: Could it actually be just a protein causing these diseases?
Speaker 2: Couldn't?
Speaker 1: Most researchers gave a firm no, but a handful we're
Speaker 1: more open minded and largely ignored Tikva Albert was in
Speaker 1: this open minded and largely ignored capillar. By way, a
Speaker 1: British mathematician actually named J. S. Griffith publishes a paper
Speaker 1: proposing several different mechanisms by which it could be a protein.
Speaker 1: But it doesn't really gain a lot of traction and
Speaker 1: the scrapy Koru krusfeldt jakub slow virus field was about
Speaker 1: to gain a new member who would give prions their
Speaker 1: name and earn a little notoriety for himself. Meet Stanley Prisoner, MD,
Speaker 1: with a background in neurology and biochemistry. Prisoner's career trajectory
Speaker 1: became a whole lot more focused when he saw his
Speaker 1: first Chrisfeldt Yakab patient in nineteen seventy two. This was
Speaker 1: a problem he knew he wanted to work on. He
Speaker 1: collaborated with the scrapy researcher William Hadlow and began attempting
Speaker 1: to purify the scrapy particle. Having that isolated agent was
Speaker 1: super important because you could do experiments directly on it
Speaker 1: to see how it would react. For instance, Prisoner and
Speaker 1: his group showed that the particle did not lose its
Speaker 1: infectivity when treated with chemicals that destroyed nucleic acids, but
Speaker 1: it did lose its infectivity when it was treated with
Speaker 1: chemicals that destroyed proteins.
Speaker 2: Oh boom, boom boom.
Speaker 1: With this purified particle, you could also design an antibody
Speaker 1: test for it, which really spread up diagnosis. Previously, you
Speaker 1: would have had to inject a lab animal with tissue
Speaker 1: from a person or sheep or whatever, and then wait
Speaker 1: for signs of disease for conformation, which can take at
Speaker 1: least a year. Yeah, the field of preon research, as
Speaker 1: it would soon be known, seems to have been filled
Speaker 1: with egos, with Prisoners perhaps the largest.
Speaker 2: Wow I'm shocked.
Speaker 1: Yeah, to say that he was not well liked by
Speaker 1: most of the field is probably understating it. Prisoner was
Speaker 1: fiercely driven, often combative, selfish with his findings, and obsessed
Speaker 1: with getting credit.
Speaker 2: God.
Speaker 1: He painted himself as a martyr and fighting the good
Speaker 1: fight against conventional thought. And part of the reason that
Speaker 1: I say this is because it was how he was
Speaker 1: written about in the Preon book that I read, which
Speaker 1: I understand is going to be It's a story, right,
Speaker 1: it comes from a certain angles biased. But I also
Speaker 1: read the book that he wrote, his memoir, and this
Speaker 1: comes through very strongly in that as well. Oh yeah, Anyway,
Speaker 1: Prisoner really did advance this field, and one way he
Speaker 1: did that was to give it its name. When he
Speaker 1: started working in this area, Kuru, Scrapie and Chris felt
Speaker 1: Yaka were all still generally referred to by the term
Speaker 1: given to it by Gadgetsek slow virus. Prisoner didn't like
Speaker 1: this name. It wasn't really accurate since no virus had
Speaker 1: been found, so he set about trying to think of
Speaker 1: a new one. He came up with Preon for a
Speaker 1: proteinaceous infectious particle, so creative props. This wasn't a popular
Speaker 1: move in the field. First of all, he was criticized
Speaker 1: for making it sound like his own name. Secondly, it
Speaker 1: dodged the question of what this particle actually was. Was
Speaker 1: a preon, a protein, a virus, or just a particle?
Speaker 2: Just a particle, man, Relax.
Speaker 1: Despite the disdain for this word, it worked, The press
Speaker 1: loved it, and it rolled right off the tongue. But
Speaker 1: prisoners still needed to get more evidence for the protein
Speaker 1: only hypothesis. To show that a protein could be infectious,
Speaker 1: he had to synthesize a protein and then introduce it
Speaker 1: to an animal.
Speaker 2: I'm so excited about this part because I read a
Speaker 2: little bit about it in the biology and I can't
Speaker 2: wait to hear more about it.
Speaker 1: Oh Man. I hope that I give you enough of
Speaker 1: what you're looking for. I'm not sure. So. A problem
Speaker 1: arose when they had purified this protein only to find
Speaker 1: out that it was just an ordinary protein produced by
Speaker 1: the own host.
Speaker 2: Can you imagine how bizarre that must have been. You're like, no,
Speaker 2: we really think we've got it. It's this thing what.
Speaker 1: Yeah. It called into question the entire concept of an
Speaker 1: infectious protein, oh Man, especially when he in collaboration with
Speaker 1: other labs found the location of this gene that produced
Speaker 1: the preon. Yeah, and he found that it was highly
Speaker 1: conserved across so many animal species, indicating that it was
Speaker 1: probably super important to keep right.
Speaker 2: It's this gene that like all these mammals have. That's
Speaker 2: why you get preon diseases in so many different mammals.
Speaker 1: Yeah, it must have just been like, well, this was
Speaker 1: a wild use chase. How are we so wrong? Right?
Speaker 2: Where do we go from here?
Speaker 1: Where did they go?
Speaker 2: Aaron?
Speaker 1: Okay, So, if the only function of this gene was
Speaker 1: to produce a protein that killed the animal, why would
Speaker 1: it still be around? How could it still be around?
Speaker 1: Prisoner had an answer for that too, the same one
Speaker 1: that the mathematician Griffith had proposed years earlier. A protein
Speaker 1: could have two forms, one that was normal and the
Speaker 1: other that was disease causing.
Speaker 2: I'm sorry, this is the first time that people realized
Speaker 2: that proteins could misfold was because of preons.
Speaker 1: I don't know, but that is so cool. Yeah, I
Speaker 1: think so. I think it is because at this point
Speaker 1: this was still just a concept. It had not been
Speaker 1: observed or demonstrated that proteins could do this. Oh, my gosh,
Speaker 1: there needed to be an experiment. Yeah, and there actually
Speaker 1: kind of was one that nature had already set up. So,
Speaker 1: like you talked about, some prion diseases can be inherited,
Speaker 1: like khruslat Yaka disease and GSS, and those diseases show
Speaker 1: that there were mutations in the prion gene leading to
Speaker 1: a state of disease. So prisoner created mice with mutations
Speaker 1: in this gene. He observed them getting sick, killed them,
Speaker 1: and then took the infectious prions from them and injected
Speaker 1: them into mice that didn't have the mutation and those
Speaker 1: got sick. And yeah, so you could see easily how
Speaker 1: a protein could be normal or infectious.
Speaker 2: Oh, my gracious.
Speaker 1: But but there still remained the mystery of the sporadic case.
Speaker 2: Yeah.
Speaker 1: So krusladyaka, like you said, could be infectious as had
Speaker 1: been demonstrated, it could be inherited as it often was,
Speaker 1: but sometimes it would just appear randomly. Yeah, this sporadic
Speaker 1: form of the disease was a bit harder to explain.
Speaker 1: If someone had a normal, non disease causing form of
Speaker 1: the preon gene, how could the disease suddenly develop prisoner
Speaker 1: once again had an answer, conformational influence. This doesn't fully
Speaker 1: explain it, really, but this is basically the concept of
Speaker 1: the domino effect of one misfolded protein causing all the
Speaker 1: other ones that came into contact with to readjust themselves.
Speaker 1: But this completed the three components of infectious, familial, and
Speaker 1: sporadic to create this unified preon protein only hypothesis.
Speaker 2: I mean, it still doesn't explain how the first one
Speaker 2: becomes misfolded sporadically in a.
Speaker 1: Person unless it's just probability.
Speaker 2: Right, exactly, Yeah, because then it's just if just randomly
Speaker 2: it might get misfolded.
Speaker 3: Yeah.
Speaker 1: But oh man, it's yeah. I know that we keep
Speaker 1: saying this, but prions are super bizarre, and it's it's
Speaker 1: no surprise that they remained such a mystery for so long,
Speaker 1: and it's really it's more incredible that some of their
Speaker 1: secrets have been revealed at all. I fee, yeah, I'm
Speaker 1: also presenting this research as though it was accepted as
Speaker 1: a fact as it happened, but in reality, it was
Speaker 1: and continues to be debated. There are still researchers who
Speaker 1: firmly believe that there is a tiny virus hiding out
Speaker 1: that will one day be uncovered. Yeah, but Prisoner was
Speaker 1: awarded the Nobel Prize for a new biological principle of
Speaker 1: infection in nineteen ninety seven. When you hear the word prion,
Speaker 1: what's the first disease that comes to your mind?
Speaker 2: Mad cow?
Speaker 1: Of course, of course it is. Mad cow disease reared
Speaker 1: its ugly head in the late nineteen eighties in Britain
Speaker 1: and flashed across headlines for years. It awakened the public
Speaker 1: to tragic failures in agricultural and public health oversight. It
Speaker 1: resulted in the deaths of hundreds of people and almost
Speaker 1: a million cattle, and it showed how much we still
Speaker 1: don't know about preons. It started, at least in the
Speaker 1: nineteen seventies. Normally docile cows started acting aggressively and had
Speaker 1: trouble walking. Death was always the outcome, Like scrapy infected sheep,
Speaker 1: when you stroked a cow's back, it would uncontrollably nibble
Speaker 1: and lipsmack. But the connection between scrapy and this new
Speaker 1: disease wouldn't be made until nineteen eighty six. Wow, a
Speaker 1: veterinary pathologist checks out some brains of a cow that
Speaker 1: had been acting like it had scrapy. According to another vet.
Speaker 1: Sure enough, this brain looks like scrapy in a cow,
Speaker 1: and scrapie in a cow is huge news. Initially, the
Speaker 1: authorities weren't that worried about the human risk. Scrapey had
Speaker 1: never been shown to be transmitted from sheep to human,
Speaker 1: but this might not follow that same pattern. One worrying
Speaker 1: finding was that it had jumped species A Niala I
Speaker 1: don't know if I'm saying that right, which is an
Speaker 1: antelope like animal, and a local zoo had died of
Speaker 1: a scrapy like disease. Bovine's bonge of form encephalopathy, which
Speaker 1: is the mouthful of a name given to the disease,
Speaker 1: started appearing all over England, and the hunt was on
Speaker 1: for the common exposure, though quietly, of course, mustn't worry
Speaker 1: the public, that's good. The Ministry of Agriculture, Fisheries and
Speaker 1: Food was housed under one roof, bureaucratically speaking, and they
Speaker 1: were in charge of both the as the name suggests,
Speaker 1: the agricultural sector and human food safety, which kind of
Speaker 1: seems like an inherent conflict of interest. They could either
Speaker 1: keep the mad cow meat out of the human food
Speaker 1: supply and economically devastate the farmers, or keep it in
Speaker 1: and potentially expose millions of humans to an incurable disease.
Speaker 1: The silence about the potential danger to humans didn't last long,
Speaker 1: nor did the original name. It was soon dubbed mad
Speaker 1: cow disease much more memorable.
Speaker 2: So much more memorable.
Speaker 1: At this time, mad cow meat was still making its
Speaker 1: way into the markets.
Speaker 2: Mad cow meat, Like, how could you call it anything
Speaker 2: but my cow?
Speaker 1: After that? If your cow died in the pasture, you
Speaker 1: couldn't sell it for human consumption. But oh no, if
Speaker 1: you got a sick cow to a slaughterhouse before it died,
Speaker 1: that was fine.
Speaker 2: Oh no.
Speaker 1: Screenings found evidence of mad cow in farms all over England.
Speaker 1: Where was it coming from? The short answer, cake in parlor?
Speaker 1: What cake in parlor?
Speaker 2: Like, we're having cake in the PARLYA dear? That was bad?
Speaker 2: It precisely okay.
Speaker 1: Cake was a high protein concentrate that farmers would give
Speaker 1: to cows who were milking in parlor. So in parlor
Speaker 1: means milking.
Speaker 2: Oh, this is cows eating cake in the parlor, not humans.
Speaker 1: Yeah, where was it coming from? In the cows got it?
Speaker 1: The protein in this cake didn't come from soy.
Speaker 2: Oh no, oh no.
Speaker 1: It generally came from farm animals that couldn't be sold
Speaker 1: to the human market. Yeah, just all bits ground up
Speaker 1: and molded into a cake.
Speaker 2: I'm sorry, I'm thinking about my dog food right now. Yeah,
Speaker 2: gross man.
Speaker 1: This explained how the disease was showing up all over
Speaker 1: the country and how the nyala had gotten sick. The
Speaker 1: zoo had recently switched from soy to meat pellets.
Speaker 2: Oh my gosh, guys, cows are not carnivores.
Speaker 1: Nope, but once they start being fed it, they they
Speaker 1: don't do well without it.
Speaker 2: They go mad for it.
Speaker 1: They go mad for it. Nerd alert.
Speaker 2: Yeah, I pushed my glasses up.
Speaker 1: Despite this very clear evidence of how preons could be
Speaker 1: introduced to cows through their food, it would be eight
Speaker 1: eight whole years until animal protein and cowfeed would be banned.
Speaker 1: The author of this book that family who Couldn't Sleep,
Speaker 1: compared it to keeping the Broad Street water pump operating
Speaker 1: for eight years after John Snow showed it was the
Speaker 1: source of cholera.
Speaker 2: We all know how bad that would have been.
Speaker 1: Yeah, it was like, that's an analogy. I can get everyone.
Speaker 2: Listen to episode four if you haven't already, and then
Speaker 2: you'll get it.
Speaker 1: Too, nice plug. Thanks. During those eight years, certain protective
Speaker 1: measures were enacted. For instance, any cows showing signs of
Speaker 1: bovine sponge offormencephalopathy were to be reported and killed with
Speaker 1: full compensation. Schools banned British beef. Many people stopped eating it,
Speaker 1: but other worrying things started to emerge. A cat nicknamed
Speaker 1: Mad Max was diagnosed with feline sponge offormencephalopathy. I think
Speaker 1: he was Max, and then he got named Mad Max.
Speaker 2: That's the cutest and saddest thing.
Speaker 1: Yeah, cows were still getting sick after Mad Kalmi was
Speaker 1: banned from the cowfood supply. Two marmosets began showing signs
Speaker 1: of the disease after being experimentally inoculated, demonstrating that the
Speaker 1: disease could be spread to primates. Big news there, and finally, tragically,
Speaker 1: human teenagers started to be diagnosed with krusfeld Yakub disease.
Speaker 1: This was not the sporadic form. This was a new variant,
Speaker 1: a cluster marked by a common exposure. Europe banned British beef.
Speaker 1: Millions of cattle were slaughtered. Estimates of the number of
Speaker 1: people who had developed this new krusfeld Yakub variant ranged
Speaker 1: from the hundreds to the millions. As of this year
Speaker 1: twenty eighteen, only two hundred and thirty one cases of
Speaker 1: the disease have been reported, but screenings of tissues from
Speaker 1: healthy people in the UK have revealed evidence of prion
Speaker 1: infection and at least four thousand people. Wow, But it's
Speaker 1: not really clear whether these people will eventually develop the
Speaker 1: disease because there's still so much not known about the
Speaker 1: tipping point from infection to disease, right, but because the
Speaker 1: story is still unfolding. And didn't even mean to make
Speaker 1: that pun.
Speaker 2: That was a protein joke, guys.
Speaker 1: And because the UK received such an enormous dose of prions,
Speaker 1: a lot of countries have regulations against blood donations by
Speaker 1: people who lived in the UK during the nineteen eighties
Speaker 1: and nineteen nineties. Yeah, mad cow has also popped up
Speaker 1: elsewhere in the world, including the US. Many mysteries remain
Speaker 1: about the mad cow disease outbreak. For instance, why England
Speaker 1: howard cows still getting sick? Proteins are really tough things.
Speaker 1: Even preon ash is apparently infectious, sure, And why did
Speaker 1: only a handful of people get sick. The answer to
Speaker 1: that last question might be in our There is variation
Speaker 1: and susceptibility to preon diseases not just among people, but
Speaker 1: also among animals like sheep and scrapy resistant breeds. Molecular
Speaker 1: research has shown that those who have two copies of
Speaker 1: the mutated gene are more likely to get the disease,
Speaker 1: while those who have one normal and one mutated copy
Speaker 1: of the gene never get the disease but can act
Speaker 1: as a carrier. Nearly all of those who developed variant
Speaker 1: krusfeld yakub from infected beef had two copies of this
Speaker 1: mutated gene. More recent research shows that in the four
Speaker 1: A people, a new variation in the prion protein emerged
Speaker 1: super recently, maybe ten generations ago, and those with this
Speaker 1: variation have resistance to KUU. That's genetic evolution. That's happening
Speaker 1: on a scale we can observe.
Speaker 2: So cool.
Speaker 1: Yeah. The final chapter almost done of preon history. I
Speaker 1: want to cover is a short one and one that
Speaker 1: will take us back to the present, to the forests
Speaker 1: of North America where thousands of deer and elk and
Speaker 1: moose are dying of chronic wasting disease, yet another preon disease.
Speaker 1: Where did this one come from? As usual, the answer
Speaker 1: isn't entirely clear, but we may have a guess. In
Speaker 1: the nineteen sixties, young biologist Jeane Schoonveld decided to study
Speaker 1: starvation and mule deer in Fort Collins, Colorado, Laura and
Speaker 1: Rick Okay to Laura, Schoonveld kept some starving deer in
Speaker 1: one pen and controlled deer in another pen so he
Speaker 1: could compare the two. This control pen also happened to
Speaker 1: house sheep. Ooh. Now, there isn't universal agreement on this,
Speaker 1: but several people on the project say that scrapie was
Speaker 1: present in those sheep. If that were the case, scrapie
Speaker 1: could have passed from the sheep to the deer who
Speaker 1: began showing signs of disease. Wow. After the experiment was over,
Speaker 1: Schuenveld released the control deer, so the ones who had
Speaker 1: been in the enclosures with the sheep back into the wild.
Speaker 1: Ten years later, researchers started noticing scrapy like behavior and
Speaker 1: brain damage in wild deer populations in Colorado and Wyoming.
Speaker 1: Since then, it has spread every year throughout the American
Speaker 1: West Plains and more recently the Midwest and Canada, Saskatchewan
Speaker 1: and Alberta. Chronic wasting may pose a threat to other
Speaker 1: ungulate species as well. It's been spoted in an elk, moose, reindeer, etc.
Speaker 1: And to other parts of the world. It's been seen
Speaker 1: in South Korea, Norway and Finland, for instance. How is
Speaker 1: it spreading? Deer aren't forced to eat deer meat, and
Speaker 1: they aren't in as high of population densities as sheep.
Speaker 1: Prions are incredibly tough, so it's proba that chronic wasting
Speaker 1: is transmitted among these ungulate species by direct contact via
Speaker 1: bodily fluids, indirect contact through a contaminated environment, or by
Speaker 1: a crow acting as a vector for dispersal of the preon.
Speaker 2: Weird a crow?
Speaker 1: Yeah, so a crow, I don't know. Basically, a crow
Speaker 1: eats a bit of this is this is in terms
Speaker 1: of long distance dispersal.
Speaker 2: I don't know.
Speaker 1: The crow eats a bit of a deer that died
Speaker 1: of chronic wasting. The preon survives the digestive journey and
Speaker 1: then it poops it out to contamine it a different
Speaker 1: part of the forest.
Speaker 2: Seems a little sas, but I mean.
Speaker 1: I'm not sure i'd yeah, but this is still an
Speaker 1: area of very active and interesting research.
Speaker 2: People at Illinois doing cool research on chronic wasting disease.
Speaker 1: Yeah. The whole time I was researching preons, I kept
Speaker 1: getting this feeling that I was just seeing the tip
Speaker 1: of the iceberg. We obviously know so much more about
Speaker 1: preons today than fifty years ago, but so much seems
Speaker 1: left to be uncovered. It truly is a new frontier
Speaker 1: of health and medical research, and it's changed the way
Speaker 1: we think about infection. Like we talked about prions don't
Speaker 1: have this biological imperative to pass down their genes. It's
Speaker 1: just a protein, and yet it is an infectious disease
Speaker 1: and one that has told us a lot about ourselves.
Speaker 1: So what I want to know is A, what do
Speaker 1: the numbers look like today? And B what's the latest
Speaker 1: on cures or treatments or technologies or whatever.
Speaker 2: Yeah, Okay, it's interesting that you described this as like
Speaker 2: a new frontier of research kind of because even in
Speaker 2: just hearing what you were just talking about with chronic
Speaker 2: wasting and what I've read about what's going on in
Speaker 2: research today, it seems like there's a lot of open
Speaker 2: territory and possibilities for research. If I wanted to keep
Speaker 2: doing research, maybe I'd switched to preons right now. But
Speaker 2: in terms of the research that's going on, it seems like,
Speaker 2: and I'm probably missing a lot of it, but it
Speaker 2: seems like there's kind of two main branches, because there's
Speaker 2: two big areas that we still lack a lot of
Speaker 2: information on. One of those areas is focused on actually
Speaker 2: understanding the mechanism behind this path of physiology of disease,
Speaker 2: and a lot of that has to do with figuring
Speaker 2: out what this protein does in our body when it's
Speaker 2: not misfolded. So because we still don't entirely know what
Speaker 2: this normal protein does. It's a protein that is very
Speaker 2: abundant in neuron cell plasma membranes, and there's a lot
Speaker 2: of thought that it has to do with possibly calcium channels,
Speaker 2: possibly copper, maybe bringing things into cells or pushing ions
Speaker 2: out of cells. It's not entirely clear. It does seem
Speaker 2: to be a really important protein, and it makes sense
Speaker 2: that it's in such high density on your neurons since
Speaker 2: those seem to be so highly affected. But we still
Speaker 2: don't know, Like there is no clear answer as to
Speaker 2: what this protein does. The other field of research is
Speaker 2: what do we do about it? We know that this
Speaker 2: disease exists, so how do we treat it?
Speaker 3: And yeah, yeah, So at this point it doesn't seem
Speaker 3: like much of that research in that realm is out
Speaker 3: of the screening phase, or maybe at best the animal
Speaker 3: model's phase. So a tiny background on drug development. Any
Speaker 3: drug that gets developed, especially if it's a brand new
Speaker 3: drug and not a drug that we use for a
Speaker 3: different disease that you want to use to treat some
Speaker 3: other disease, it goes through a lot of stages of screening.
Speaker 3: Usually it's tested in cell culture first to make sure
Speaker 3: it does the thing that you're trying to get it
Speaker 3: to do, and then you screen it. You often screen
Speaker 3: a whole bunch of different compounds and you say, okay,
Speaker 3: which one of these compounds has an effect on this
Speaker 3: protein or against this pathogen. So there's a lot of papers,
Speaker 3: especially from the early two thousands, where they were screening
Speaker 3: hundreds or sometimes thousands of compounds to see if any
Speaker 3: of them had an effect on diminishing the accumulation of
Speaker 3: misfolded preon proteins. Okay, So there was at least some
Speaker 3: work that has been done so far and is i'm
Speaker 3: sure continuing to be done to try and just find compounds.
Speaker 3: Once they find those promising compounds, then you test those
Speaker 3: on animals, usually in mice, but also in other animals
Speaker 3: as well to see if they're safe and if they
Speaker 3: actually work once you take them out of a cell
Speaker 3: culture and put them into a real animal. Then once
Speaker 3: you can show that a compound actually works on a
Speaker 3: disease in an animal, you have to test it on
Speaker 3: small groups of humans to make sure that it doesn't
Speaker 3: kill people or cause any other serious reactions. And that's
Speaker 3: a phase one trial, and then you do a phase
Speaker 3: two trial, which is a larger group of humans, and
Speaker 3: now you want to look at does it actually work.
Speaker 3: Like we know it doesn't kill you, now we can
Speaker 3: ask does it actually work? Then you have to do
Speaker 3: even bigger trials. So it's a very very long process
Speaker 3: before you actually get any sort of drug to market,
Speaker 3: and as far as I can tell, any potential drugs
Speaker 3: seem to be in the very early stages. Still.
Speaker 2: There are some drugs or some treatments that have been
Speaker 2: used to treat other diseases. There's an anahistamine which is
Speaker 2: super random. Anahistamine is like benadryl. It's like what you
Speaker 2: take if you have allergies or something like that. Yeah,
Speaker 2: that in those early trials of trying to identify compounds,
Speaker 2: seemed to have effect on binding to Alzheimer's proteins, misfolded
Speaker 2: Alzheimer's proteins, so it was suggested that perhaps it also
Speaker 2: could bind too misfolded preon proteins. Oh, it has been
Speaker 2: shown in cell culture also to inhibit the preon protein,
Speaker 2: but I haven't I haven't found any papers that it
Speaker 2: has been tested on beyond the cell culture level. But
Speaker 2: that's a drug that already exists and has been used
Speaker 2: in humans. It's not generally used because there's a lot
Speaker 2: of side effects. But you know, whenever we look at drugs,
Speaker 2: we have to look at the side effects versus the disease.
Speaker 2: And if you're treating something as benign as allergies, you
Speaker 2: want to make sure you have very little side effects.
Speaker 2: But if you're treating something where the only alternative is death,
Speaker 2: you might tolerate a lot more side effects. So who knows.
Speaker 2: There's another treatment called PPS, which is pentocin polysulfate that
Speaker 2: it's been getting some press and trying to treat preon diseases,
Speaker 2: but it has not been shown to work. So people
Speaker 2: have actually tried this in at least a couple of
Speaker 2: humans and they died. It didn't work at all.
Speaker 1: So but it's still getting pressed.
Speaker 2: It's still getting press, or at least it got press.
Speaker 2: I don't know if it's still getting press. There's at
Speaker 2: least one study that suggests that it delays the progression
Speaker 2: of disease and decreases the amount of abnormal protein deposition
Speaker 2: in mice brains, but you had to inject it directly
Speaker 2: into the mouse, so that's not likely something that's going
Speaker 2: to be in human trial soon. But yeah, yeah, So
Speaker 2: I don't know. It seems like there's a lot of
Speaker 2: open territory, and I do think that a lot of
Speaker 2: this kind of hinges on being able to figure out
Speaker 2: this protein. Because with a lot of other diseases, even
Speaker 2: if we don't fully understand everything about a bacterium or
Speaker 2: a virus, we know generally how they replicate, We know
Speaker 2: generally you know how they operate, and so we can
Speaker 2: use drugs that we have to try and stop them.
Speaker 2: There are targets that we know that we can aim
Speaker 2: for But with this, it's it's really hard and it is,
Speaker 2: like you said early on, it's a protein that's in
Speaker 2: our bodies, and presumably it's an important one, you know, right,
Speaker 2: it has to be. Yeah, In terms of numbers, the
Speaker 2: only one where there's you can get sort of good
Speaker 2: numbers on is classic cruise felt yakup disease. And so
Speaker 2: the majority of those cases eighty five percent are sporadic,
Speaker 2: about five to fifteen percent are inherited, and overall those
Speaker 2: are at a rate of about one to three cases
Speaker 2: per million people per year.
Speaker 1: Oh, it's quite rare. Okay, Yeah, it's pretty rare.
Speaker 2: So in the US, for example, there have been eleven
Speaker 2: thousand cases reported from nineteen seventy nine to twenty sixteen,
Speaker 2: so between yeah, between one hundred and eighty and four
Speaker 2: hundred cases per year. And when you look so on
Speaker 2: the CDC's website, you can find a chart essentially of
Speaker 2: the number of cases that have been reported in the
Speaker 2: US each year of classic CJD, and the numbers get
Speaker 2: bigger every year. But it's important to keep in mind
Speaker 2: that it could be in part due to better screening
Speaker 2: and recognition. It also could be because our population size
Speaker 2: has grown a lot since nineteen seventy nine. So when
Speaker 2: you see four hundred verses one twenty, it's sometimes easy
Speaker 2: to go, oh my god, it's growing, and it's but
Speaker 2: it actually has been at a pretty constant rate.
Speaker 1: I've also read that a lot of or at least
Speaker 1: like a proportion of CJD patients have been misdiagnosed as Alzheimer's.
Speaker 2: Absolutely yeah, because with CJD you present with similar symptoms.
Speaker 2: You present with, you know, rapid onset dementia. I think
Speaker 2: the difference is the onset of time and then the
Speaker 2: time to death. Alzheimer's is a very very slow progressing disease.
Speaker 2: Generally it's faster if it's earlier onset. This is what
Speaker 2: I want to end on.
Speaker 1: Okay.
Speaker 2: I think that chronic wasting disease is so important, and
Speaker 2: I hope that I know that there are so many
Speaker 2: people studying it, but I I just want them to
Speaker 2: get so much money for research because be cuse so
Speaker 2: there have been no there has been so far no
Speaker 2: evidence that chronic wasting disease in deer can cause disease
Speaker 2: in humans. There have been at least a few studies
Speaker 2: that have looked for those links. Because here's the thing
Speaker 2: about chronic wasting. Even though we haven't yet found any
Speaker 2: evidence that chronic wasting disease in deer can cause disease
Speaker 2: in humans, it has been found at very high prevalence
Speaker 2: in deer populations. In wild deer populations rates up to
Speaker 2: ten or twenty five percent, but in captive herds up
Speaker 2: to eighty percent of deer have been found to be infected.
Speaker 2: And if you combine that with data from the CDC,
Speaker 2: that suggests that up to twenty percent of people surveyed
Speaker 2: had hunted deer at some point, and up to two
Speaker 2: thirds of Americans have eaten venice.
Speaker 1: In if it I've eaten venison, I've eaten venison.
Speaker 2: Our neighbor hunts deer, breaks them over, and there's chronic
Speaker 2: wasting disease in Illinois. I find the prospect terrifying.
Speaker 1: Yeah, tip of the iceberg, Tip of the iceberg.
Speaker 2: It's so interesting because it's not outside the rum of possibility. Right,
Speaker 2: we know that it can happen from eating contaminated cow meat,
Speaker 2: right because I don't think there have been any cases
Speaker 2: of humans getting any kind of preon disease from eating
Speaker 2: sheep meat infected with scrapy. Is that correct?
Speaker 1: That's what it seems to be.
Speaker 2: Yes, And so I'm curious if maybe it's because the
Speaker 2: outbreaks of variant CJD that were from mad cow disease.
Speaker 2: I wonder if it's just that the prions were in
Speaker 2: higher concentration in the cow because of the ways that
Speaker 2: the cows got infected, so then now they're in higher
Speaker 2: concentrations in humans.
Speaker 1: I don't know.
Speaker 2: I don't think anybody knows.
Speaker 1: Right something about the folding of that particular fold. Who knows?
Speaker 2: Yeah, right, yeah, who knows? I want to know, though.
Speaker 1: I know, I know, yeah, you know. And kind of
Speaker 1: in retrospect, despite that being one of the longest histories
Speaker 1: that we've ever discussed, it feels premature because it seems
Speaker 1: like there's so much more to be done and that
Speaker 1: there's so much more that's going to be discovered.
Speaker 2: Yeah, And I feel like in talking about what's currently happening,
Speaker 2: it's kind of just like so many things, but I
Speaker 2: don't know how to talk about any of them because
Speaker 2: they're all kind of in their infancy, like there's no
Speaker 2: you know, there's no here's the new drug or here's
Speaker 2: we have an answer. It's all just currently being figured out,
Speaker 2: which I find so exciting.
Speaker 1: Yeah, it's super super like cutting edge. Yeah, news is
Speaker 1: happening right.
Speaker 2: Yeah. On that note, should we share our sources?
Speaker 1: Yeah, great idea. So I read a couple of books.
Speaker 1: One is called Madness and Memory and that is by
Speaker 1: Stanley Prusner, so that is his memoir of pre on research.
Speaker 1: I also read The Family That Couldn't Sleep by Dtmax,
Speaker 1: great overview of preon history. And then I have a
Speaker 1: few articles which I will put on our sources list.
Speaker 1: And then finally, if you're interested, there is a documentary
Speaker 1: about Carlton Gadgetsek called The Genius and the Boys.
Speaker 2: I have so many articles they're all going to be
Speaker 2: posted on our website, this podcast will Kill You dot
Speaker 2: Com under the episodes tab, so we post all of
Speaker 2: our sources from every single episode, this one in particular.
Speaker 2: I have a lot of cool articles about the current
Speaker 2: research that's being done.
Speaker 1: So if you're interested.
Speaker 2: In reading more about what's going on with pre on research, now,
Speaker 2: definitely check that out. Cool. Yeah, Well, thank you everyone
Speaker 2: for listening. We couldn't make a podcast without you. Well
Speaker 2: we could, but then it would be just us, be weird.
Speaker 1: It would be weird. Thanks to Bloodmobile for providing the
Speaker 1: music for this and all of our episodes.
Speaker 2: Thanks thanks so much.
Speaker 1: Well, on that note, wash your hands
Speaker 2: You filthy animals.