WEBVTT - Ep 20 Prions: Apocalypse Cow

0:00:01.160 --> 0:00:04.440
<v Speaker 1>Typically one day in middle age, the sufferer finds that

0:00:04.480 --> 0:00:07.560
<v Speaker 1>he has begun to sweat. A look in the mirror

0:00:07.600 --> 0:00:10.160
<v Speaker 1>will show that his pupils have shrunk to pinpricks, and

0:00:10.240 --> 0:00:12.760
<v Speaker 1>he is holding his head in an odd, stiff way.

0:00:13.520 --> 0:00:17.159
<v Speaker 1>Constipation is common. The women suddenly enter menopause and the

0:00:17.200 --> 0:00:20.880
<v Speaker 1>men become impotent. The sufferer begins to have trouble sleeping

0:00:20.960 --> 0:00:24.040
<v Speaker 1>and tries compensating with a nap in the afternoon, but

0:00:24.120 --> 0:00:27.440
<v Speaker 1>to no avail. His blood pressure and pulse have become

0:00:27.480 --> 0:00:32.280
<v Speaker 1>elevated and his body is an overdrive. Over the ensuing months,

0:00:32.320 --> 0:00:35.519
<v Speaker 1>he tries desperately to sleep, sometimes closing his eyes, but

0:00:35.640 --> 0:00:38.400
<v Speaker 1>never succeeding in falling into more than a light stupor.

0:00:39.040 --> 0:00:44.120
<v Speaker 1>Their exhaustion is immense, beyond comprehension. Once the sufferer can

0:00:44.159 --> 0:00:48.040
<v Speaker 1>no longer sleep, a downward progression ensues as he loses

0:00:48.040 --> 0:00:52.440
<v Speaker 1>his ability to walk or balance. Perhaps most tragic, the

0:00:52.479 --> 0:00:56.040
<v Speaker 1>ability to think remains intact. Sufferers know what is happening.

0:00:56.560 --> 0:00:58.600
<v Speaker 1>At first, they can talk about it and even write

0:00:58.640 --> 0:01:02.080
<v Speaker 1>down their thoughts. After a few more months, some lose

0:01:02.120 --> 0:01:06.199
<v Speaker 1>this level of functioning once their bodies shut down. Only

0:01:06.240 --> 0:01:08.600
<v Speaker 1>the desperate look in their eyes shows that they know

0:01:08.720 --> 0:01:11.840
<v Speaker 1>what is going on, but others can talk and reason

0:01:11.920 --> 0:01:15.759
<v Speaker 1>until the end. In the terminal phase, usually about fifteen

0:01:15.800 --> 0:01:19.000
<v Speaker 1>months after the disease has begun, they fall into a

0:01:19.040 --> 0:01:57.320
<v Speaker 1>state of exhaustion resembling a coma and die. Yeah.

0:01:57.400 --> 0:01:59.600
<v Speaker 2>God, that's a bad one.

0:02:00.960 --> 0:02:04.520
<v Speaker 1>Yeah. Do you want to hear what that is? Yeah,

0:02:04.560 --> 0:02:10.079
<v Speaker 1>so that is a case of fatal familial insomnia, which

0:02:10.320 --> 0:02:15.520
<v Speaker 1>is a preon disease. Welcome to preons everyone, And that

0:02:15.720 --> 0:02:18.320
<v Speaker 1>is an excerpt from the book The Family Who Couldn't

0:02:18.360 --> 0:02:21.040
<v Speaker 1>Sleep by DT Max, which is all about preons.

0:02:21.560 --> 0:02:22.040
<v Speaker 2>Wow.

0:02:22.880 --> 0:02:26.959
<v Speaker 1>Yes, hi, Hi, I'm Aaron.

0:02:26.760 --> 0:02:29.600
<v Speaker 2>Welsh and I'm Erin alman Updyke.

0:02:29.720 --> 0:02:31.800
<v Speaker 1>And this is this podcast will kill you.

0:02:32.680 --> 0:02:35.200
<v Speaker 2>Yeah, today we're talking about preons.

0:02:37.320 --> 0:02:40.160
<v Speaker 1>This is a big one. And we've said that before.

0:02:41.400 --> 0:02:45.639
<v Speaker 2>We say that almost every time. Yeah, but it's always true,

0:02:45.919 --> 0:02:49.680
<v Speaker 2>it is. Yeah, we haven't really covered any like baby diseases.

0:02:49.280 --> 0:02:54.320
<v Speaker 1>Yet before we get into the nitty gritty of these

0:02:54.919 --> 0:02:58.680
<v Speaker 1>many different diseases. What time is.

0:02:58.639 --> 0:03:00.560
<v Speaker 2>It, it's quarantine any time.

0:03:02.840 --> 0:03:04.240
<v Speaker 1>What are we drinking today?

0:03:04.400 --> 0:03:12.600
<v Speaker 2>Well, this week we're drinking the chronically wasted because one

0:03:12.600 --> 0:03:16.240
<v Speaker 2>of the prion diseases is chronic wasting disease.

0:03:17.880 --> 0:03:27.560
<v Speaker 1>It has milk naturally, bourbon naturally, coffee naturally, no, just

0:03:27.600 --> 0:03:30.520
<v Speaker 1>because those things taste good together, and then a little

0:03:30.560 --> 0:03:33.000
<v Speaker 1>bit of cinnamon and nutmeg.

0:03:33.080 --> 0:03:34.960
<v Speaker 2>And you just shake it all up and then strain

0:03:35.040 --> 0:03:39.200
<v Speaker 2>it into a glass. And as always this season, we'll

0:03:39.240 --> 0:03:43.480
<v Speaker 2>also be posting this recipe along with our place burrita,

0:03:43.600 --> 0:03:47.320
<v Speaker 2>the non alcoholic version of our quarantines, on all of

0:03:47.320 --> 0:03:51.240
<v Speaker 2>our social media accounts and our website, So check it

0:03:51.240 --> 0:03:51.680
<v Speaker 2>out there.

0:03:52.880 --> 0:03:59.520
<v Speaker 1>Excellent, Okay, preons. I have read so much about the history,

0:03:59.600 --> 0:04:03.480
<v Speaker 1>about the whatever, but I really avoided the biology and

0:04:03.600 --> 0:04:06.720
<v Speaker 1>understanding exactly what's going on, So please enlighten me.

0:04:33.120 --> 0:04:39.279
<v Speaker 2>So, prions are a pathogen unlike any that we've ever

0:04:39.320 --> 0:04:43.360
<v Speaker 2>looked at before, and unlike anything that we'll ever look

0:04:43.440 --> 0:04:46.120
<v Speaker 2>at again, which is kind of thrilling.

0:04:47.360 --> 0:04:48.040
<v Speaker 1>It really is.

0:04:48.360 --> 0:04:53.240
<v Speaker 2>So every other infectious disease that we've discussed so far

0:04:54.200 --> 0:04:57.479
<v Speaker 2>and that will ever discuss again has been either a

0:04:57.560 --> 0:05:01.640
<v Speaker 2>virus or a bacterium or protozoan. In the future, will

0:05:01.640 --> 0:05:05.080
<v Speaker 2>probably do a bunch of worms. But even at their

0:05:05.120 --> 0:05:09.159
<v Speaker 2>most basic, all of these infectious diseases are some kind

0:05:09.200 --> 0:05:13.240
<v Speaker 2>of organism which I'm putting in quotes because some people

0:05:13.279 --> 0:05:17.440
<v Speaker 2>don't call viruses organisms, but at a bare minimum, even viruses,

0:05:17.839 --> 0:05:23.800
<v Speaker 2>they've got either RNA or DNA and protein, and the

0:05:23.880 --> 0:05:28.320
<v Speaker 2>genetic material is required because basically that's the only way

0:05:28.760 --> 0:05:32.839
<v Speaker 2>that an organism can reproduce. The DNA or the RNA

0:05:33.040 --> 0:05:36.960
<v Speaker 2>serve as templates to actually make the protein, and so

0:05:37.040 --> 0:05:40.719
<v Speaker 2>then they also serve as the template that's replicated to

0:05:40.880 --> 0:05:45.279
<v Speaker 2>make a new version of that organism. So for anything

0:05:45.360 --> 0:05:48.520
<v Speaker 2>to be considered even close to a live and for

0:05:48.560 --> 0:05:52.280
<v Speaker 2>sure to be able to reproduce, it has to have

0:05:52.440 --> 0:05:56.960
<v Speaker 2>either DNA or RNA.

0:05:55.680 --> 0:05:59.560
<v Speaker 1>Except for prerias.

0:06:02.520 --> 0:06:10.960
<v Speaker 2>I'm excited. So preons are just protein, no nucleic acids,

0:06:11.680 --> 0:06:17.600
<v Speaker 2>no genetic material, no DNA, no RNA, just protein. The

0:06:17.640 --> 0:06:25.599
<v Speaker 2>word preon literally comes from protonaceous infectious particle. So creative.

0:06:26.200 --> 0:06:32.720
<v Speaker 1>I it's it's so amazing to me because there's no

0:06:33.960 --> 0:06:35.719
<v Speaker 1>biological incentive.

0:06:37.000 --> 0:06:39.400
<v Speaker 2>Oh my god, you're still right.

0:06:40.160 --> 0:06:44.000
<v Speaker 1>I don't get it. I don't get it. How why?

0:06:44.600 --> 0:06:46.760
<v Speaker 2>Okay, I'm gonna try and answer it those two questions.

0:06:48.800 --> 0:06:51.320
<v Speaker 2>As you can imagine, you are not the only person

0:06:51.440 --> 0:06:53.919
<v Speaker 2>to feel like this, like what, how? Why? And I

0:06:53.960 --> 0:06:57.080
<v Speaker 2>can't wait to hear the story, the story of how

0:06:57.720 --> 0:07:01.880
<v Speaker 2>just mind blown scientists must have been when they finally

0:07:01.960 --> 0:07:08.520
<v Speaker 2>were accepting that this was just a protein. It's oh,

0:07:08.640 --> 0:07:10.680
<v Speaker 2>there's nothing like it. Okay, So here's how it goes.

0:07:11.680 --> 0:07:15.160
<v Speaker 2>We're going to talk about proteins for a minute. Imagine

0:07:15.680 --> 0:07:20.160
<v Speaker 2>that a protein is basically a rope. Okay. There are

0:07:20.240 --> 0:07:23.320
<v Speaker 2>lots of different material that you can use to make rope,

0:07:23.720 --> 0:07:26.200
<v Speaker 2>and there's lots of different lengths to which you can

0:07:26.240 --> 0:07:29.680
<v Speaker 2>cut that rope. Just like protein in our body. We

0:07:29.760 --> 0:07:32.680
<v Speaker 2>have lots and lots of different types of proteins. They're

0:07:32.680 --> 0:07:35.560
<v Speaker 2>all made up of different amino acids, and they're all

0:07:35.560 --> 0:07:38.720
<v Speaker 2>different lengths and sizes, and they all have different functions,

0:07:39.720 --> 0:07:43.080
<v Speaker 2>and a large part of how that function is determined

0:07:43.200 --> 0:07:47.119
<v Speaker 2>is how they're folded, or, if you're imagining our protein rope,

0:07:47.440 --> 0:07:51.360
<v Speaker 2>how they're nottted. Okay, So think of how many different

0:07:51.400 --> 0:07:57.000
<v Speaker 2>ways you can tie a knot. There are thousands, hundreds probably,

0:07:59.200 --> 0:08:01.239
<v Speaker 2>And if you think of all of the different types

0:08:01.280 --> 0:08:04.080
<v Speaker 2>of materials of rope, some of them are going to

0:08:04.080 --> 0:08:09.880
<v Speaker 2>be better at holding certain knots than others. So proteins, yeah, right,

0:08:10.040 --> 0:08:13.040
<v Speaker 2>this is a good analogy. I didn't come up with it,

0:08:13.080 --> 0:08:19.960
<v Speaker 2>Brett did. So different materials of rope or different types

0:08:20.000 --> 0:08:23.240
<v Speaker 2>of proteins are only going to be stable and therefore

0:08:23.320 --> 0:08:28.480
<v Speaker 2>functional in certain conformations. Certain knots with me so far?

0:08:28.880 --> 0:08:32.760
<v Speaker 2>Oh yeah, great. So in the case of preon diseases,

0:08:33.360 --> 0:08:40.000
<v Speaker 2>there's this single protein, this single rope. It's called PRP,

0:08:40.520 --> 0:08:44.559
<v Speaker 2>which just means get ready for the creativity here protein

0:08:44.800 --> 0:08:51.160
<v Speaker 2>preon protein, no joke. So this protein is in your body.

0:08:51.200 --> 0:08:53.760
<v Speaker 2>It's in your body, it's in my body, it's in

0:08:53.800 --> 0:08:58.040
<v Speaker 2>everyone's who's listening's body. It's a cell membrane protein, which

0:08:58.080 --> 0:09:01.040
<v Speaker 2>means it's found in the walls of your cells, in

0:09:01.080 --> 0:09:03.800
<v Speaker 2>a whole bunch of our different cells, especially in our neurons.

0:09:04.520 --> 0:09:09.199
<v Speaker 2>And we're not entirely clear what this protein does exactly.

0:09:09.320 --> 0:09:12.839
<v Speaker 2>We don't know its exact function. We think it has

0:09:12.880 --> 0:09:16.640
<v Speaker 2>to do with neuronal communication and the transport of stuff

0:09:16.679 --> 0:09:19.199
<v Speaker 2>inside and outside the cell. That doesn't really matter right

0:09:19.240 --> 0:09:23.760
<v Speaker 2>now for my purposes. The thing that matters is that

0:09:23.840 --> 0:09:28.680
<v Speaker 2>this protein is normal most of the time, and when

0:09:28.800 --> 0:09:32.880
<v Speaker 2>it's in its normal state, it's normal, not it's benign.

0:09:33.120 --> 0:09:38.240
<v Speaker 2>It doesn't cause any disease. But for some reason, if

0:09:38.240 --> 0:09:43.960
<v Speaker 2>it unknots itself and then renots itself in an abnormal way.

0:09:44.080 --> 0:09:49.200
<v Speaker 2>It can begin to cause disease. And here's the scary part.

0:09:50.200 --> 0:09:54.440
<v Speaker 2>When it interacts with other normal proteins in your body,

0:09:54.600 --> 0:09:58.400
<v Speaker 2>it can cause them to change shape. And then those

0:09:58.520 --> 0:10:03.079
<v Speaker 2>newly misfolded proteins interact with other normal proteins and cause

0:10:03.160 --> 0:10:07.400
<v Speaker 2>them to change shape. And then do you see where

0:10:07.440 --> 0:10:08.200
<v Speaker 2>I'm going with this.

0:10:09.280 --> 0:10:14.200
<v Speaker 1>It's a domino effect of terrifying misfolded proteins.

0:10:14.360 --> 0:10:22.000
<v Speaker 2>That's exactly what it is, and it's outrageous. It's similar

0:10:22.120 --> 0:10:25.679
<v Speaker 2>in some ways to how a virus or a bacterium

0:10:26.040 --> 0:10:29.280
<v Speaker 2>will get into your body and replicate and grow in number.

0:10:29.320 --> 0:10:32.640
<v Speaker 2>But in this case, it's just a misfolded version of

0:10:32.679 --> 0:10:36.920
<v Speaker 2>a protein that's already in your body, and in its

0:10:36.960 --> 0:10:39.040
<v Speaker 2>normal form, it's not a big deal. But if it

0:10:39.080 --> 0:10:43.439
<v Speaker 2>comes into contact with a misfolded version, it becomes misfolded

0:10:43.480 --> 0:10:48.680
<v Speaker 2>and that's how it replicates. And exactly how this happens

0:10:48.679 --> 0:10:55.640
<v Speaker 2>to begin with, we don't know. Down Damn, I'm just struggling,

0:10:56.320 --> 0:10:57.719
<v Speaker 2>struggling for words right now.

0:10:57.760 --> 0:11:02.199
<v Speaker 1>I'm struggling for words because it is. It sounds like

0:11:02.240 --> 0:11:03.400
<v Speaker 1>it's from a sci fi book.

0:11:03.840 --> 0:11:05.040
<v Speaker 2>Yeah, it totally does.

0:11:05.600 --> 0:11:09.400
<v Speaker 1>And it's it's just very it's it's outside of this

0:11:09.400 --> 0:11:15.840
<v Speaker 1>this paradigm of infection or disease or proteins that we have,

0:11:15.960 --> 0:11:20.720
<v Speaker 1>all that we have been taught throughout school, throughout life. Whatever.

0:11:21.920 --> 0:11:24.240
<v Speaker 2>Yeah, we kind of mentioned this at the beginning, but

0:11:25.000 --> 0:11:28.000
<v Speaker 2>there are a lot of different names for preon diseases.

0:11:28.520 --> 0:11:31.600
<v Speaker 2>But the thing that I did not realize until starting

0:11:31.600 --> 0:11:34.560
<v Speaker 2>to research for this episode is that all of these

0:11:34.559 --> 0:11:38.680
<v Speaker 2>different diseases that you've probably heard of are all caused

0:11:38.800 --> 0:11:44.080
<v Speaker 2>by misfolding of the same protein, the same one, that

0:11:44.280 --> 0:11:48.240
<v Speaker 2>PRP protein. It's not tons of different proteins in a

0:11:48.240 --> 0:11:52.040
<v Speaker 2>cow versus a sheep versus a human, It's the same one.

0:11:52.280 --> 0:11:59.400
<v Speaker 2>How how how great question. Honestly, I did not know

0:11:59.480 --> 0:12:03.840
<v Speaker 2>that at all. Now. The thing is, each of these misfoldings,

0:12:03.880 --> 0:12:08.599
<v Speaker 2>these different knots in the rope, they're slightly different versions,

0:12:08.960 --> 0:12:11.520
<v Speaker 2>so they kind of refer to them as different strains,

0:12:11.559 --> 0:12:14.520
<v Speaker 2>the way that we would call different strains of ebola virus.

0:12:14.720 --> 0:12:17.120
<v Speaker 2>It's the same virus, but there are multiple strains. It's

0:12:17.120 --> 0:12:20.160
<v Speaker 2>similar in this except that, again, it's just a protein,

0:12:20.960 --> 0:12:26.280
<v Speaker 2>but they're all misfolded in slightly different ways. So these

0:12:26.320 --> 0:12:31.760
<v Speaker 2>different variants of the misfolding of the preon protein. They

0:12:31.800 --> 0:12:36.800
<v Speaker 2>cause different diseases that were named by and classified by

0:12:36.840 --> 0:12:42.960
<v Speaker 2>their incubation period, which varies really widely, and the actual neuropathology.

0:12:43.040 --> 0:12:45.080
<v Speaker 2>So the way that they affect your brain and the

0:12:45.120 --> 0:12:48.320
<v Speaker 2>symptoms that they cause are actually a little different in

0:12:48.440 --> 0:12:53.000
<v Speaker 2>each version of preon disease, even though it's the same

0:12:53.200 --> 0:12:56.840
<v Speaker 2>misfolded protein. So what I'm going to do is go

0:12:56.920 --> 0:12:59.360
<v Speaker 2>through the different human diseases, and I am going to

0:12:59.360 --> 0:13:02.240
<v Speaker 2>focus on human diseases, but we can talk about some

0:13:02.360 --> 0:13:06.000
<v Speaker 2>of the other mammal diseases too, because they're also really interesting.

0:13:06.480 --> 0:13:07.560
<v Speaker 1>Don't worry, I'll get there.

0:13:07.640 --> 0:13:11.920
<v Speaker 2>Oh good, So we'll go through the different human ones,

0:13:12.600 --> 0:13:14.240
<v Speaker 2>but first I just want to talk to you about

0:13:14.240 --> 0:13:17.960
<v Speaker 2>what they all have in common. So the other name

0:13:18.000 --> 0:13:26.400
<v Speaker 2>for preon diseases is transmissible sponge ofform encephalopathy, which janus

0:13:29.000 --> 0:13:31.800
<v Speaker 2>so what does that mean? It means this transmissible just

0:13:31.840 --> 0:13:34.199
<v Speaker 2>tells us that this is an infectious disease, meaning it

0:13:34.240 --> 0:13:36.520
<v Speaker 2>can be passed from one person to another, or in

0:13:36.559 --> 0:13:40.840
<v Speaker 2>some cases, from one mammal to another. Incephalopathy just means

0:13:40.840 --> 0:13:43.800
<v Speaker 2>it affects your brain and sponge ofform tells us how

0:13:43.880 --> 0:13:48.840
<v Speaker 2>it affects your brain. It actually causes spongeiform changes, so

0:13:49.160 --> 0:13:55.839
<v Speaker 2>your brain becomes wholly like a sponge. And importantly, this

0:13:56.320 --> 0:14:01.040
<v Speaker 2>these holes that happen, it happens without any inflammation. So

0:14:01.160 --> 0:14:04.440
<v Speaker 2>inflammation is a response of our body. It's a natural

0:14:04.480 --> 0:14:07.840
<v Speaker 2>response to either tissue damage or the presence of some

0:14:07.920 --> 0:14:11.960
<v Speaker 2>kind of non self like bacteria or viruses. But in

0:14:12.000 --> 0:14:15.280
<v Speaker 2>this case, with preons, your body doesn't react to the

0:14:15.320 --> 0:14:19.040
<v Speaker 2>preon proteins or to the damage they cause with normal

0:14:19.160 --> 0:14:24.280
<v Speaker 2>inflammatory responses. So you have neurons and other brain cells

0:14:24.480 --> 0:14:30.000
<v Speaker 2>actively dying, leaving gaping holes empty space in their place,

0:14:30.360 --> 0:14:34.080
<v Speaker 2>with no white blood cell invasion, no activation of the

0:14:34.160 --> 0:14:38.880
<v Speaker 2>inflammatory cascade or anything like that. So that's what they

0:14:38.920 --> 0:14:44.360
<v Speaker 2>have in common. Let's talk briefly about the different variance.

0:14:43.920 --> 0:14:44.640
<v Speaker 1>Of this disease.

0:14:45.360 --> 0:14:47.880
<v Speaker 2>Okay, so the first one, since we touched on it

0:14:47.920 --> 0:14:53.080
<v Speaker 2>briefly in our first hand account, is fatal familial insomnia. Now,

0:14:53.120 --> 0:14:57.400
<v Speaker 2>this is a weird one. It's super super super rare,

0:14:57.720 --> 0:15:02.800
<v Speaker 2>like very rare, but it causes insomnia, that's one of

0:15:02.840 --> 0:15:07.000
<v Speaker 2>the major symptoms. It also causes speech and physical coordination

0:15:07.120 --> 0:15:14.640
<v Speaker 2>problems and dementia. This one is not necessarily infectious, but

0:15:14.760 --> 0:15:20.000
<v Speaker 2>it is familial, so it's actually transmitted genetically. And it's

0:15:20.040 --> 0:15:22.920
<v Speaker 2>autismal dominant, which means that if one of your parents

0:15:23.040 --> 0:15:25.640
<v Speaker 2>has it, you have a fifty percent chance of getting

0:15:25.680 --> 0:15:26.160
<v Speaker 2>it as.

0:15:26.040 --> 0:15:27.760
<v Speaker 1>Well, like Huntington's.

0:15:27.880 --> 0:15:33.440
<v Speaker 2>Exactly, just like Huntington's. So it's a weird one. Okay,

0:15:33.760 --> 0:15:36.640
<v Speaker 2>the next one, we're just gonna blow through these. The

0:15:36.680 --> 0:15:43.120
<v Speaker 2>next one is called GSS Gershmann Straussler Schineker syndrome. Was

0:15:43.160 --> 0:15:46.600
<v Speaker 2>that okay, I don't know. I don't know either. I

0:15:46.640 --> 0:15:51.960
<v Speaker 2>don't speak German. This is another weird one. It's also familial,

0:15:52.600 --> 0:15:59.440
<v Speaker 2>so just like fatal familial insomnia, it doesn't cause insomnia though. Instead,

0:15:59.560 --> 0:16:03.360
<v Speaker 2>what happened is you first start with dysarthria, which means

0:16:03.400 --> 0:16:06.120
<v Speaker 2>difficulty speaking, so you might not be able to talk.

0:16:07.120 --> 0:16:11.240
<v Speaker 2>And then you'll get what's called a taxia, which means

0:16:11.320 --> 0:16:14.840
<v Speaker 2>you're you can't coordinate your body movements, so you might

0:16:14.960 --> 0:16:19.960
<v Speaker 2>have tremors, you might have an unsteady, wobbling gait. And

0:16:20.040 --> 0:16:25.720
<v Speaker 2>then you'll progress to dementia, memory loss, visual disturbances, and

0:16:26.280 --> 0:16:27.120
<v Speaker 2>eventually death.

0:16:28.840 --> 0:16:30.920
<v Speaker 1>That sounds like it's going to be the common thread,

0:16:30.960 --> 0:16:32.160
<v Speaker 1>and I know that it is, but.

0:16:32.520 --> 0:16:36.160
<v Speaker 2>It is the other thing that I didn't mention that

0:16:36.400 --> 0:16:40.440
<v Speaker 2>ties all of these together is that they are one fatal.

0:16:41.080 --> 0:16:43.920
<v Speaker 1>Cool. Yeah, so that's our second this season.

0:16:45.000 --> 0:16:49.040
<v Speaker 2>Yeah. Well, I mean Raby's was like almost one hundred percent.

0:16:50.760 --> 0:16:53.920
<v Speaker 2>At least you can treat it. If you can't, Yeah,

0:16:55.040 --> 0:16:55.880
<v Speaker 2>we've got to have one.

0:16:56.680 --> 0:16:57.960
<v Speaker 1>Yeah.

0:16:58.120 --> 0:17:00.920
<v Speaker 2>Now, let's get into the diseases. You've probably heard more

0:17:00.920 --> 0:17:03.880
<v Speaker 2>about kuru Have you heard of it?

0:17:04.520 --> 0:17:05.000
<v Speaker 1>Oh?

0:17:05.200 --> 0:17:09.000
<v Speaker 2>Well, I know you have listeners. Have you tell us?

0:17:10.000 --> 0:17:13.800
<v Speaker 2>Kourou is a disease that is transmissible and it's believed

0:17:13.920 --> 0:17:17.520
<v Speaker 2>to have been transmitted to people from the consumption of

0:17:18.000 --> 0:17:22.240
<v Speaker 2>deceased family members during burial rituals, which I'm guessing you're

0:17:22.280 --> 0:17:25.040
<v Speaker 2>going to talk a lot more about, Aaron. Oh yeah,

0:17:25.119 --> 0:17:28.040
<v Speaker 2>I avoided the history of this. What I do know

0:17:28.520 --> 0:17:30.919
<v Speaker 2>is that this was one of one of the early

0:17:31.240 --> 0:17:36.560
<v Speaker 2>preon diseases that people sort of found out about, right, Yeah,

0:17:36.600 --> 0:17:39.760
<v Speaker 2>And one of the first ways that we figured out

0:17:39.800 --> 0:17:42.879
<v Speaker 2>that this was actually a transmissible disease and how people

0:17:42.880 --> 0:17:46.480
<v Speaker 2>actually got it from eating meat that had been contaminated

0:17:46.560 --> 0:17:51.320
<v Speaker 2>with preon proteins. So for this one, the symptoms start

0:17:51.520 --> 0:17:56.119
<v Speaker 2>often with body tremors, so all over body tremors and

0:17:56.160 --> 0:18:01.960
<v Speaker 2>then bursts of laughter, which I find so interesting and

0:18:02.160 --> 0:18:06.600
<v Speaker 2>scary because what was it was described as people would

0:18:06.640 --> 0:18:10.200
<v Speaker 2>become very depressed, which is understandable, but then they would

0:18:10.240 --> 0:18:13.640
<v Speaker 2>have these bursts of uncontrollable laughter that they just couldn't

0:18:13.960 --> 0:18:14.719
<v Speaker 2>couldn't stop.

0:18:15.280 --> 0:18:15.840
<v Speaker 1>Yeah.

0:18:16.119 --> 0:18:19.679
<v Speaker 2>Yeah. Then you would also get that same ataxia, so

0:18:20.200 --> 0:18:23.960
<v Speaker 2>tremors combined with a wobbly gait and just not being

0:18:23.960 --> 0:18:27.280
<v Speaker 2>able to coordinate your body movements. And then in the

0:18:27.359 --> 0:18:31.440
<v Speaker 2>later stages you can actually get ulcerations, so open wounds,

0:18:31.680 --> 0:18:34.439
<v Speaker 2>like on various places of your body that lead to

0:18:34.520 --> 0:18:39.359
<v Speaker 2>secondary infection. Oh yeah, and so actually one of the

0:18:39.400 --> 0:18:43.160
<v Speaker 2>major causes of death in Kuru is pneumonia or another

0:18:43.280 --> 0:18:47.920
<v Speaker 2>secondary infection, not necessarily the preon proteins itself.

0:18:48.400 --> 0:18:49.679
<v Speaker 1>Interesting. I didn't know that.

0:18:50.040 --> 0:18:55.840
<v Speaker 2>Yeah huh. And then we have the most famous or

0:18:55.880 --> 0:18:59.800
<v Speaker 2>should I say actually the two most famous probably for

0:19:00.400 --> 0:19:06.119
<v Speaker 2>of preon disease in humans krutz feld yakup disease also

0:19:07.600 --> 0:19:12.280
<v Speaker 2>variant kruzfeld yacup disease. There are two there's actually more

0:19:12.280 --> 0:19:14.520
<v Speaker 2>than two forms, but these two forms are very distinct.

0:19:14.600 --> 0:19:21.480
<v Speaker 2>So let's talk about the classic. First, classic CJD is sporadic.

0:19:22.080 --> 0:19:24.840
<v Speaker 2>This is not what you get from eating cow meat

0:19:25.080 --> 0:19:29.080
<v Speaker 2>that's been contaminated. This is not mad cow. So krutzveeld

0:19:29.160 --> 0:19:33.280
<v Speaker 2>yakup disease is a preon disease that we don't know

0:19:34.080 --> 0:19:37.840
<v Speaker 2>why or how it happens. We think maybe it's just

0:19:38.040 --> 0:19:43.840
<v Speaker 2>age related random mutations. Aw who knows. You can get them.

0:19:45.880 --> 0:19:49.560
<v Speaker 2>You can get them from things like corneal transplants. There

0:19:49.600 --> 0:19:52.760
<v Speaker 2>have been several documented cases of people who received cornea

0:19:52.800 --> 0:19:57.639
<v Speaker 2>transplants from someone who had documentedly who had died from

0:19:58.080 --> 0:20:01.720
<v Speaker 2>kruzfeld yakup disease, who then got krus folt yacka disease

0:20:01.760 --> 0:20:05.600
<v Speaker 2>and died from the cornea transplant, and also dura mater transplants.

0:20:05.640 --> 0:20:09.399
<v Speaker 2>Dura mater is the outer layer of your brain mi ninja,

0:20:09.560 --> 0:20:13.800
<v Speaker 2>so like the tissue that covers your brain. Essentially okay,

0:20:14.160 --> 0:20:16.280
<v Speaker 2>Sometimes you might do a graft of that from one

0:20:16.320 --> 0:20:19.000
<v Speaker 2>person to another in the case of some extreme trauma,

0:20:19.680 --> 0:20:22.560
<v Speaker 2>and if that person died from krus folt yakub, then

0:20:22.640 --> 0:20:28.480
<v Speaker 2>you will also. So this type of CJD is characterized

0:20:28.520 --> 0:20:32.640
<v Speaker 2>by a rapidly progressive dementia and memory loss. Those are

0:20:32.680 --> 0:20:35.399
<v Speaker 2>the first two things that tend to happen, and then

0:20:35.480 --> 0:20:38.840
<v Speaker 2>you'll get personality changes hallucinations.

0:20:40.400 --> 0:20:44.359
<v Speaker 1>So when you say rapidly progressive, what's what's the timeframe

0:20:44.359 --> 0:20:45.040
<v Speaker 1>we're looking at.

0:20:45.200 --> 0:20:47.520
<v Speaker 2>So most of the time that was something I didn't

0:20:47.520 --> 0:20:49.439
<v Speaker 2>mention about all of these diseases, but they all have

0:20:49.600 --> 0:20:54.080
<v Speaker 2>very different durations of illness. So fatal familial insomnia, the

0:20:54.160 --> 0:20:58.520
<v Speaker 2>average age at death is fifty and the average duration

0:20:58.600 --> 0:21:01.000
<v Speaker 2>of illness, so from the time you start showing symptoms

0:21:01.119 --> 0:21:05.720
<v Speaker 2>until you die, is eighteen months for f FI. Okay,

0:21:06.040 --> 0:21:12.240
<v Speaker 2>for Gershmann, Straussler, Shinecker, GSS, the median age at death

0:21:12.280 --> 0:21:15.240
<v Speaker 2>it's really variable because it's such a small population, but

0:21:15.680 --> 0:21:19.399
<v Speaker 2>the duration of illness is anywhere from three months to

0:21:19.520 --> 0:21:22.359
<v Speaker 2>thirteen years, with an average of five years.

0:21:22.600 --> 0:21:23.200
<v Speaker 1>So too much.

0:21:23.240 --> 0:21:27.800
<v Speaker 2>God, Yeah, it's a very long progressing disease. Kuru, the

0:21:27.920 --> 0:21:31.680
<v Speaker 2>duration of illness is only about twelve months, but they

0:21:31.680 --> 0:21:34.120
<v Speaker 2>think that the incubation period could be as long as

0:21:34.320 --> 0:21:38.800
<v Speaker 2>fifty years. That's the time from when you first get

0:21:39.040 --> 0:21:42.439
<v Speaker 2>exposed to prion's until they build up in enough number

0:21:42.560 --> 0:21:46.840
<v Speaker 2>in your brain to actually start causing disease. But with

0:21:46.960 --> 0:21:51.520
<v Speaker 2>Krutzfelt Yakub classic CJD. The median age at death is sixty,

0:21:51.880 --> 0:21:54.679
<v Speaker 2>but the duration of illness is only four to six months,

0:21:56.080 --> 0:21:59.840
<v Speaker 2>So once you start showing symptoms, you're probably dead within

0:21:59.840 --> 0:22:00.560
<v Speaker 2>five months.

0:22:00.960 --> 0:22:01.760
<v Speaker 1>Holy cow.

0:22:02.320 --> 0:22:05.320
<v Speaker 2>The longest that's been documented that somebody has survived after

0:22:05.359 --> 0:22:10.879
<v Speaker 2>starting to show symptoms is about two years with classic CJD. Wow. Yeah,

0:22:10.920 --> 0:22:15.960
<v Speaker 2>so very rapidly progressive. You'll also end up with hallucinations,

0:22:16.080 --> 0:22:19.960
<v Speaker 2>mild clonus, which means muscle spasms, and then late in

0:22:20.000 --> 0:22:22.960
<v Speaker 2>the disease you can have things like a taxia again,

0:22:23.040 --> 0:22:26.359
<v Speaker 2>that wobbly gait, So we're seeing some overlap with symptoms

0:22:26.560 --> 0:22:30.439
<v Speaker 2>speech impairment again. Here the cause of death tends to

0:22:30.480 --> 0:22:34.399
<v Speaker 2>be pneumonia, which I find very interesting, especially that it

0:22:34.400 --> 0:22:36.560
<v Speaker 2>can happen that rapidly.

0:22:36.880 --> 0:22:40.439
<v Speaker 1>Well, and also I don't understand the mechanism behind, Like

0:22:40.520 --> 0:22:43.919
<v Speaker 1>this is a neurological disorder, Tell me about it.

0:22:44.320 --> 0:22:48.320
<v Speaker 2>Yeah, so, how how well if you're if it's affecting

0:22:48.359 --> 0:22:51.120
<v Speaker 2>the parts of your brain that cause you to breathe normally,

0:22:51.440 --> 0:22:53.119
<v Speaker 2>and if you're not breathing normally, or if you're not

0:22:53.160 --> 0:22:56.080
<v Speaker 2>coughing up normally, or if it's even just affecting like

0:22:56.359 --> 0:22:58.800
<v Speaker 2>all of the functions of your organs, right, everything is

0:22:58.840 --> 0:23:01.439
<v Speaker 2>controlled by your brain. It's maybe impairing more of our

0:23:01.520 --> 0:23:05.840
<v Speaker 2>unconscious functions than it is our conscious functions. Okay, and

0:23:05.880 --> 0:23:09.080
<v Speaker 2>then the last human preon disease that we need to

0:23:09.119 --> 0:23:14.879
<v Speaker 2>talk about is variant Krutzfeld yakup. This is the one

0:23:15.080 --> 0:23:21.400
<v Speaker 2>associated with mad cow. So this is the one which

0:23:21.440 --> 0:23:25.600
<v Speaker 2>I'm sure you'll talk about the outbreak that started it all,

0:23:26.000 --> 0:23:29.200
<v Speaker 2>don't you know it where a bunch of people became infected,

0:23:29.440 --> 0:23:37.399
<v Speaker 2>presumably after eating contaminated cow meat beef that had those

0:23:37.520 --> 0:23:41.280
<v Speaker 2>cows had died from mad cow, also known as bovine

0:23:41.440 --> 0:23:45.520
<v Speaker 2>sponge offm encephalopathy or BSE. So that is the form

0:23:45.720 --> 0:23:49.720
<v Speaker 2>of preon disease that happens in cows, and if you

0:23:49.760 --> 0:23:52.879
<v Speaker 2>eat a cow that's died from that, you will get

0:23:53.200 --> 0:23:58.200
<v Speaker 2>v CJD, very different from classic CJD. The median age

0:23:58.240 --> 0:24:01.920
<v Speaker 2>at death is thirty or under, so these were young

0:24:02.040 --> 0:24:06.280
<v Speaker 2>people who are affected, and the median duration of illness

0:24:06.400 --> 0:24:09.920
<v Speaker 2>is thirteen to fourteen months. So it's a longer duration

0:24:10.080 --> 0:24:11.040
<v Speaker 2>of disease.

0:24:11.080 --> 0:24:14.320
<v Speaker 1>Hm hm, possibly because it came from.

0:24:15.800 --> 0:24:19.080
<v Speaker 2>Cal Yeah, yeah, so it it presents in a way

0:24:19.119 --> 0:24:22.480
<v Speaker 2>that's much more similar to KURU, and that's part of

0:24:22.520 --> 0:24:24.280
<v Speaker 2>the reason that they were able to make that link

0:24:24.320 --> 0:24:31.000
<v Speaker 2>between vCJD and KURU both being transmitted by consuming preon

0:24:31.240 --> 0:24:38.240
<v Speaker 2>infected meats, because they do present very similarly. With v CJD.

0:24:38.440 --> 0:24:42.000
<v Speaker 2>It's hard acronym to say, you present not so much

0:24:42.040 --> 0:24:45.119
<v Speaker 2>with dementia and memory loss like with classic Chris felt Yaka,

0:24:45.160 --> 0:24:49.840
<v Speaker 2>but with other psychiatric and behavioral symptoms, and also very

0:24:49.880 --> 0:24:55.720
<v Speaker 2>classically with very painful what's called diastesias, which means nerve

0:24:55.960 --> 0:24:59.560
<v Speaker 2>pain and like your nerves are firing in ways that

0:24:59.640 --> 0:25:02.520
<v Speaker 2>is really painful and not under your control, so maybe

0:25:02.520 --> 0:25:07.719
<v Speaker 2>you'll have hands cramping and things like that. Yeah, you

0:25:07.800 --> 0:25:13.919
<v Speaker 2>don't get as much of the ataxias and things with

0:25:14.119 --> 0:25:17.280
<v Speaker 2>v CJD as you do with some of the other diseases,

0:25:18.480 --> 0:25:21.800
<v Speaker 2>but you do die just like with all the others.

0:25:22.240 --> 0:25:25.160
<v Speaker 2>And one of the scary things about vCJD is that

0:25:25.280 --> 0:25:28.720
<v Speaker 2>it is believed that it can also be transmitted by

0:25:28.800 --> 0:25:36.320
<v Speaker 2>blood products. So that means like blood products, what do

0:25:36.400 --> 0:25:41.240
<v Speaker 2>you that's another way to say that sirum serum. Yeah,

0:25:41.440 --> 0:25:44.920
<v Speaker 2>if you give blood transfusion, That's what I'm trying to say.

0:25:46.600 --> 0:25:49.679
<v Speaker 2>Whereas there isn't really evidence that other forms of this

0:25:49.760 --> 0:25:54.320
<v Speaker 2>disease can be transmitted in that way. Oh yeah. So,

0:25:54.480 --> 0:25:58.600
<v Speaker 2>while you can get CJD from corneo graphs or dural

0:25:58.640 --> 0:26:01.160
<v Speaker 2>graphs or things where you're actually coming in contact with

0:26:01.560 --> 0:26:05.240
<v Speaker 2>neuronal surfaces, there is an evidence that you can get

0:26:05.280 --> 0:26:11.119
<v Speaker 2>it from blood products. But with v CJD you can

0:26:11.400 --> 0:26:15.000
<v Speaker 2>potentially transmit that because you do find preon proteins in

0:26:15.040 --> 0:26:18.920
<v Speaker 2>the peripheral blood, not just in the nervous system.

0:26:19.640 --> 0:26:24.959
<v Speaker 1>That's very strange, yes, huh yeah.

0:26:25.280 --> 0:26:29.000
<v Speaker 2>And the other thing that's similar between KURU and vCJD

0:26:29.600 --> 0:26:33.280
<v Speaker 2>is that you get more deposition of the actual preon

0:26:33.320 --> 0:26:37.480
<v Speaker 2>proteins into plaques, which means that these misfold proteins aggregate,

0:26:37.800 --> 0:26:40.960
<v Speaker 2>clump together, and deposit into your brain in a similar

0:26:41.000 --> 0:26:45.000
<v Speaker 2>way that you get aggregates in something like Alzheimer's. And

0:26:45.160 --> 0:26:47.680
<v Speaker 2>it's also we have no idea how long the incubation

0:26:47.840 --> 0:26:50.600
<v Speaker 2>period is for these two diseases. These are the most

0:26:50.680 --> 0:26:55.040
<v Speaker 2>sort of transmissible of the transmissible sponge ofform and cephalopathies

0:26:55.080 --> 0:26:59.480
<v Speaker 2>in humans, and we don't know how long ago some

0:26:59.520 --> 0:27:01.320
<v Speaker 2>people or infected.

0:27:01.359 --> 0:27:02.000
<v Speaker 1>It could be.

0:27:02.080 --> 0:27:04.320
<v Speaker 2>In some cases, it seems to be a matter of months.

0:27:04.320 --> 0:27:06.879
<v Speaker 2>In other cases it could be twenty thirty, forty years.

0:27:07.359 --> 0:27:09.840
<v Speaker 2>So a lot of people think that they are going

0:27:09.880 --> 0:27:12.439
<v Speaker 2>to be more and more cases of vCJD down the

0:27:12.480 --> 0:27:15.480
<v Speaker 2>line from people who were exposed back in the nineties.

0:27:16.080 --> 0:27:17.160
<v Speaker 1>That's alarming.

0:27:17.480 --> 0:27:23.200
<v Speaker 2>It's alarming, and probably the most alarming thing about preons

0:27:23.440 --> 0:27:29.000
<v Speaker 2>is that we can't do anything about them. So there's

0:27:29.040 --> 0:27:30.760
<v Speaker 2>no treatment, is it?

0:27:30.800 --> 0:27:33.879
<v Speaker 1>Because it's a protein that's in your body, and so

0:27:33.920 --> 0:27:37.120
<v Speaker 1>by attacking that protein, you're liable to attack other proteins

0:27:37.160 --> 0:27:38.439
<v Speaker 1>that you actually need and function.

0:27:38.880 --> 0:27:41.720
<v Speaker 2>It's part of it. Yeah, But I mean cancer cells

0:27:41.720 --> 0:27:43.720
<v Speaker 2>are also your body cells, but we have ways that

0:27:43.760 --> 0:27:46.760
<v Speaker 2>we can kill those. The reason it's so hard to

0:27:46.880 --> 0:27:51.800
<v Speaker 2>deal with preons is because most of the drugs that

0:27:51.840 --> 0:27:56.159
<v Speaker 2>we use to target diseases, infectious diseases and things like

0:27:56.200 --> 0:28:02.840
<v Speaker 2>cancers target DNA A target reproduction in the way that

0:28:02.880 --> 0:28:07.399
<v Speaker 2>we know that reproduction happens. We don't know why this

0:28:07.520 --> 0:28:11.920
<v Speaker 2>protein becomes misfolded, and we don't know how it causes

0:28:12.040 --> 0:28:16.120
<v Speaker 2>other proteins to become misfolded, so we don't know how

0:28:16.160 --> 0:28:18.920
<v Speaker 2>to target it. And because we don't know exactly what

0:28:19.000 --> 0:28:23.680
<v Speaker 2>the PRP protein does normally, it makes it even harder

0:28:23.720 --> 0:28:28.359
<v Speaker 2>to actually find ways to try and stop it from

0:28:28.400 --> 0:28:31.640
<v Speaker 2>misfolding or you know, make it go back to its

0:28:31.640 --> 0:28:36.720
<v Speaker 2>normal confirmation, Yeah, is there.

0:28:36.760 --> 0:28:41.000
<v Speaker 1>Is there at least promising work on stuff like how

0:28:41.040 --> 0:28:44.800
<v Speaker 1>it's analogous to Alzheimer's or is that telling us anything

0:28:44.800 --> 0:28:47.080
<v Speaker 1>more about these other neurodegenerative diseases.

0:28:47.360 --> 0:28:49.160
<v Speaker 2>It's a good question. We'll talk a little bit more

0:28:49.160 --> 0:28:51.640
<v Speaker 2>about it in this sort of current events section. There's

0:28:51.680 --> 0:28:54.440
<v Speaker 2>definitely a lot of parallels, especially in terms of understanding

0:28:54.520 --> 0:28:59.520
<v Speaker 2>how proteins fold and why they misfold in certain ways.

0:29:00.080 --> 0:29:03.000
<v Speaker 2>These don't tend to be misfolded, at least from what

0:29:03.160 --> 0:29:06.120
<v Speaker 2>I read, they don't tend to be misfolded in the

0:29:06.160 --> 0:29:11.160
<v Speaker 2>same ways that Alzheimer's proteins are misfolded. So it's not

0:29:11.200 --> 0:29:16.840
<v Speaker 2>the same conformations that cause Alzheimer's plaques, if that makes sense.

0:29:18.760 --> 0:29:23.680
<v Speaker 2>But yeah, we don't have we don't have a lot currently,

0:29:23.720 --> 0:29:26.120
<v Speaker 2>and they're also just really hard to get rid of.

0:29:26.360 --> 0:29:32.080
<v Speaker 2>Like if your meat becomes contaminated, there's not much that

0:29:32.120 --> 0:29:36.600
<v Speaker 2>you can really do about it because proteins don't degrade

0:29:36.640 --> 0:29:39.440
<v Speaker 2>as easily as DNA or RNA. So the ways that

0:29:39.480 --> 0:29:45.080
<v Speaker 2>we normally use to sterilize. Things don't always work on preons.

0:29:45.120 --> 0:29:47.680
<v Speaker 2>They're not impossible to kill, though people who are like

0:29:48.040 --> 0:29:50.720
<v Speaker 2>you can't have a kill a preon, like that's not true.

0:29:50.760 --> 0:29:53.000
<v Speaker 2>It just takes a lot higher heat. It takes things

0:29:53.000 --> 0:29:55.880
<v Speaker 2>that dnature proteins rather things that rather than things that

0:29:56.000 --> 0:30:00.920
<v Speaker 2>dnature DNA and RNA, So it's different techniques. It is possible,

0:30:00.960 --> 0:30:02.760
<v Speaker 2>though it's not impossible.

0:30:03.400 --> 0:30:03.600
<v Speaker 1>Right.

0:30:05.840 --> 0:30:07.400
<v Speaker 2>Yeah, how's that?

0:30:07.400 --> 0:30:14.520
<v Speaker 1>That was pretty once, very scary introduction to Yeah, we

0:30:14.600 --> 0:30:15.360
<v Speaker 1>didn't even.

0:30:15.240 --> 0:30:20.360
<v Speaker 2>Talk about scrapy or BSSE or chronic wasting disease, which

0:30:20.400 --> 0:30:23.080
<v Speaker 2>I think is actually the scariest one. So Aarin, tell

0:30:23.120 --> 0:30:26.400
<v Speaker 2>me what is up with these preons?

0:30:26.720 --> 0:30:27.600
<v Speaker 1>How did they get here?

0:30:27.920 --> 0:30:31.880
<v Speaker 2>And why should I be so afraid of them?

0:30:33.440 --> 0:30:36.560
<v Speaker 1>I think that last question you're going to have to answer. Actually,

0:30:36.600 --> 0:30:38.920
<v Speaker 1>I think that last question you just answered.

0:30:39.000 --> 0:30:40.680
<v Speaker 2>Oh okay, well, then just tell me how they got

0:30:40.720 --> 0:30:42.000
<v Speaker 2>here and what's going on with them?

0:30:42.600 --> 0:31:08.000
<v Speaker 1>Okay, happy to do that. I'm mourning you.

0:31:08.080 --> 0:31:08.320
<v Speaker 2>Now.

0:31:09.320 --> 0:31:13.560
<v Speaker 1>The history of prions is a huge one, in part

0:31:13.600 --> 0:31:17.040
<v Speaker 1>because it's not really just one disease like you just

0:31:17.080 --> 0:31:21.120
<v Speaker 1>talked about. Each prion disease has its own detective story,

0:31:21.160 --> 0:31:24.000
<v Speaker 1>and each contributes to the story of prions as a whole,

0:31:25.360 --> 0:31:27.680
<v Speaker 1>and that story is going to take us all over

0:31:27.720 --> 0:31:31.200
<v Speaker 1>the world, from the pastures of Spain to the highlands

0:31:31.240 --> 0:31:34.080
<v Speaker 1>of Papua New Guinea, from the grocery stores of Britain

0:31:34.480 --> 0:31:38.120
<v Speaker 1>to the forests of North America. And we're going to

0:31:38.160 --> 0:31:42.200
<v Speaker 1>meet some rather interesting people along the way who will

0:31:42.240 --> 0:31:45.880
<v Speaker 1>show us that changing people's minds can be exceptionally hard,

0:31:46.680 --> 0:31:50.080
<v Speaker 1>especially when ego and glory seem to be driving you

0:31:50.160 --> 0:31:54.920
<v Speaker 1>more than a quest for truth. Let's get started. Even

0:31:54.960 --> 0:31:58.000
<v Speaker 1>though the word prion was only invented a few decades ago,

0:31:58.320 --> 0:32:01.240
<v Speaker 1>the history of prion disease is which is back much farther,

0:32:01.800 --> 0:32:05.760
<v Speaker 1>probably hundreds of thousands of years. But before I get

0:32:05.800 --> 0:32:09.880
<v Speaker 1>to that, let's try to track when preon diseases first

0:32:09.920 --> 0:32:14.120
<v Speaker 1>became known to modern humans. There are many early references

0:32:14.200 --> 0:32:16.920
<v Speaker 1>to a pre on like condition that have been put

0:32:16.960 --> 0:32:19.560
<v Speaker 1>forth as evidence for the disease being present at a

0:32:19.560 --> 0:32:24.440
<v Speaker 1>certain time or place. Hippocrates may have mentioned it. There's

0:32:24.560 --> 0:32:30.520
<v Speaker 1>strong support that Shakespeare refers to it in Macbeth. Yeah,

0:32:30.560 --> 0:32:33.560
<v Speaker 1>I didn't read it, but a lot of things are like,

0:32:33.600 --> 0:32:37.320
<v Speaker 1>oh yeah in Macbeth, blah blah blah, I should probably

0:32:37.320 --> 0:32:42.720
<v Speaker 1>find that line, and it was probably around during those times.

0:32:43.280 --> 0:32:45.959
<v Speaker 1>But let's get down to when it first started making

0:32:46.200 --> 0:32:50.160
<v Speaker 1>big waves. Between the eighteenth and nineteenth centuries in Europe,

0:32:50.320 --> 0:32:53.800
<v Speaker 1>human population was on the rise, and that meant figuring

0:32:53.800 --> 0:32:57.160
<v Speaker 1>out ways to make more food, to make more clothing, housing,

0:32:57.720 --> 0:33:01.640
<v Speaker 1>et cetera, to support the growing populace. And one way

0:33:01.680 --> 0:33:06.040
<v Speaker 1>that people maximized efficiency and productivity was through selective breeding,

0:33:06.640 --> 0:33:09.120
<v Speaker 1>which wouldn't actually go by that name for almost one

0:33:09.200 --> 0:33:12.040
<v Speaker 1>hundred years when Darwin would discuss it in his books,

0:33:12.920 --> 0:33:16.880
<v Speaker 1>but the concept of breeding plants or animals to select

0:33:16.880 --> 0:33:19.840
<v Speaker 1>for certain desired traits was known, and one of the

0:33:19.840 --> 0:33:24.160
<v Speaker 1>biggest developers of selective breeding was a dude named Robert Bakewell,

0:33:25.040 --> 0:33:28.240
<v Speaker 1>who applied it to sheep and in doing so changed

0:33:28.240 --> 0:33:29.320
<v Speaker 1>the course of history.

0:33:31.080 --> 0:33:33.360
<v Speaker 2>Always the bake wells with the sheep.

0:33:35.480 --> 0:33:38.640
<v Speaker 1>What is the fastest way to get the traits that

0:33:38.720 --> 0:33:40.000
<v Speaker 1>you want in a sheep?

0:33:41.560 --> 0:33:44.160
<v Speaker 2>I don't have the slightest idea.

0:33:44.360 --> 0:33:49.760
<v Speaker 1>Okay, well, you would breed the sheep who already look

0:33:49.880 --> 0:33:51.840
<v Speaker 1>the closest to your ideal image.

0:33:52.000 --> 0:33:53.040
<v Speaker 2>Okay, that makes sense.

0:33:53.160 --> 0:33:58.160
<v Speaker 1>Bakewell started doing what was known as in and in breeding.

0:33:58.760 --> 0:34:03.120
<v Speaker 1>Uh uh yeah, which means mating parents with offspring offspring

0:34:03.160 --> 0:34:06.880
<v Speaker 1>with each other. Really Game of Thrones type.

0:34:07.440 --> 0:34:10.319
<v Speaker 2>I was gonna say, just like you know England, but.

0:34:13.400 --> 0:34:13.840
<v Speaker 1>To day.

0:34:15.320 --> 0:34:15.600
<v Speaker 2>You know.

0:34:17.080 --> 0:34:19.880
<v Speaker 1>This, This practice wasn't really done because it was common

0:34:19.920 --> 0:34:22.799
<v Speaker 1>knowledge among farmers that inbred animals tended to have more

0:34:22.800 --> 0:34:26.920
<v Speaker 1>hereditary defects. But the results that Bakewell had gotten in

0:34:26.960 --> 0:34:30.040
<v Speaker 1>a really short amount of time were too impressive to ignore,

0:34:30.560 --> 0:34:33.880
<v Speaker 1>and soon enough Bakewell was studding out his best rams,

0:34:34.200 --> 0:34:37.560
<v Speaker 1>and farmers everywhere started their own in and in breeding practices.

0:34:38.960 --> 0:34:43.920
<v Speaker 1>In those years, the relatedness of those sheep sharply increased,

0:34:44.239 --> 0:34:49.399
<v Speaker 1>as you might expect, and things were about to get

0:34:49.440 --> 0:34:53.520
<v Speaker 1>a whole lot more related. English sheep weren't known for

0:34:53.560 --> 0:34:58.600
<v Speaker 1>their fleece, unlike the Spanish Merino breed.

0:34:58.920 --> 0:34:59.919
<v Speaker 2>Your favorite sheep.

0:35:00.080 --> 0:35:04.759
<v Speaker 1>I believe Marino wool is one of my absolute favorite things.

0:35:04.560 --> 0:35:05.239
<v Speaker 2>On Yeah I know.

0:35:09.600 --> 0:35:12.560
<v Speaker 1>So anyway, Yeah, so a guy named Banks decided to

0:35:12.560 --> 0:35:15.560
<v Speaker 1>turn a profit by shipping a handful of Spanish Merino

0:35:15.600 --> 0:35:19.400
<v Speaker 1>sheep to farmers all over England where they were bred

0:35:19.480 --> 0:35:21.719
<v Speaker 1>in and inn even more so they could get that

0:35:21.800 --> 0:35:25.279
<v Speaker 1>nice fleece. By the end of the seventeen hundreds, a.

0:35:26.560 --> 0:35:30.560
<v Speaker 1>Most of England's sheep were intensively in bread and b

0:35:31.080 --> 0:35:34.960
<v Speaker 1>The inbreeding was not in pockets, but rather all over.

0:35:35.280 --> 0:35:37.440
<v Speaker 1>It was the only way you could maintain sheep.

0:35:38.280 --> 0:35:38.440
<v Speaker 2>Uh.

0:35:39.600 --> 0:35:43.520
<v Speaker 1>Then a few sheep started acting strange. They seemed to

0:35:43.520 --> 0:35:45.439
<v Speaker 1>have an itch. They could not scratch enough.

0:35:46.040 --> 0:35:46.239
<v Speaker 3>OHI.

0:35:46.719 --> 0:35:49.879
<v Speaker 1>They would rub their heads and their rumps on posts,

0:35:49.880 --> 0:35:52.960
<v Speaker 1>on trees, on rocks, on fences, on anything to try

0:35:53.000 --> 0:35:55.680
<v Speaker 1>to get some kind of relief. But this was a

0:35:55.680 --> 0:35:59.640
<v Speaker 1>one direction disease. The sheep never recovered from their itch,

0:35:59.760 --> 0:36:03.359
<v Speaker 1>and later stages staggered around until they suddenly dropped dead.

0:36:04.600 --> 0:36:08.240
<v Speaker 1>Soon this phenomenon was all over England and Scotland, earning

0:36:08.320 --> 0:36:13.760
<v Speaker 1>various names along the way, such as Rubbers and Yuki Pine,

0:36:14.520 --> 0:36:24.320
<v Speaker 1>the Frenzies, the giddies, Scratchy Shrewcroft, turn sick, the disease,

0:36:25.640 --> 0:36:33.680
<v Speaker 1>the disease no like getting dizzy, Oh the dizzy.

0:36:36.200 --> 0:36:38.520
<v Speaker 2>It's like, I'm sorry, Preon is now officially the least

0:36:38.560 --> 0:36:41.799
<v Speaker 2>creative group of people. We're just gonna sell this one

0:36:41.840 --> 0:36:42.440
<v Speaker 2>of the disease.

0:36:42.680 --> 0:36:46.320
<v Speaker 1>I kind of like it. It's like the band, Yeah,

0:36:46.640 --> 0:36:53.520
<v Speaker 1>just classic, the shaking, the mad Staggers, which might be

0:36:53.560 --> 0:36:57.880
<v Speaker 1>my favorite, the goggles, and finally the one that stuck

0:36:57.920 --> 0:37:05.400
<v Speaker 1>with it. Everywhere sheep were dropping by the dozens hundreds,

0:37:05.520 --> 0:37:08.719
<v Speaker 1>and no one knew what was causing it. Hypotheses, of

0:37:08.760 --> 0:37:12.280
<v Speaker 1>course abounded. It was the air, It was a maggot,

0:37:12.320 --> 0:37:15.240
<v Speaker 1>It was too much sex, it was not enough sex.

0:37:16.200 --> 0:37:19.279
<v Speaker 1>It turned out that it had previously been described by

0:37:19.280 --> 0:37:23.720
<v Speaker 1>Spanish shepherds decades before, but this outbreak probably wasn't caused

0:37:23.760 --> 0:37:27.719
<v Speaker 1>by the import of those sheep, just a matter of

0:37:27.920 --> 0:37:31.319
<v Speaker 1>high sheep density and a lack of scrapy resistance in

0:37:31.360 --> 0:37:34.440
<v Speaker 1>the inbread sheep. No real progress would be made for

0:37:34.640 --> 0:37:38.000
<v Speaker 1>years on what the actual cause of scrapey was, but

0:37:38.160 --> 0:37:41.040
<v Speaker 1>solving that mystery lost a little bit of its urgency

0:37:41.160 --> 0:37:45.200
<v Speaker 1>as scrapy resistant sheep breeds began to take over. Wow,

0:37:46.120 --> 0:37:49.200
<v Speaker 1>let's say goodbye for now to our beautiful Merino sheep

0:37:49.280 --> 0:37:52.480
<v Speaker 1>in England and traveled to Papua New Guinea in the

0:37:52.560 --> 0:37:57.239
<v Speaker 1>nineteen fifties, which was at the time partially under Australian rule.

0:37:58.600 --> 0:38:01.120
<v Speaker 1>The Australians in charge didn't really know a lot about

0:38:01.160 --> 0:38:04.280
<v Speaker 1>the islands culture's ecology, et cetera, but they were looking

0:38:04.280 --> 0:38:07.839
<v Speaker 1>to change that. In particularly, they wanted to know who

0:38:07.920 --> 0:38:11.080
<v Speaker 1>was living in the dense rainforests of the Highlands to

0:38:11.960 --> 0:38:15.480
<v Speaker 1>make contact. I'm saying that in air quotes rather than conquer.

0:38:16.520 --> 0:38:16.840
<v Speaker 2>Yeah.

0:38:17.320 --> 0:38:22.600
<v Speaker 1>Anyway. One of the groups that was contacted was called

0:38:22.640 --> 0:38:26.400
<v Speaker 1>the Fora. Right away, those making the contact noticed a

0:38:26.440 --> 0:38:30.520
<v Speaker 1>couple of strange things. For one, there weren't many women around,

0:38:30.680 --> 0:38:34.120
<v Speaker 1>but there were a lot of unmarried men, and after

0:38:34.160 --> 0:38:36.719
<v Speaker 1>spending a bit more time with them, they noticed a

0:38:36.719 --> 0:38:40.840
<v Speaker 1>mysterious illness that was circulating, resulting in a high number

0:38:40.840 --> 0:38:44.919
<v Speaker 1>of deaths. Those afflicted, mostly women, would shiver and jerk

0:38:45.000 --> 0:38:49.080
<v Speaker 1>uncontrollably for weeks, occasionally hit as you mentioned by this

0:38:49.200 --> 0:38:54.200
<v Speaker 1>involuntary hysterical laughter, and then this would continue until they died.

0:38:55.160 --> 0:38:58.920
<v Speaker 1>The Fora called it shaking or kuru, and it was

0:38:59.160 --> 0:39:03.680
<v Speaker 1>devastating Phila. Seeing this, the Australians decided to send for

0:39:03.719 --> 0:39:07.760
<v Speaker 1>a doctor to investigate what exactly was going on. The doctor,

0:39:07.880 --> 0:39:12.600
<v Speaker 1>a man named Victor Ziegas, interviewed sufferers and took many

0:39:12.640 --> 0:39:15.600
<v Speaker 1>samples to send off for analysis, but no one could

0:39:15.600 --> 0:39:19.280
<v Speaker 1>find anything in the blood or serum or even brain samples.

0:39:19.280 --> 0:39:23.640
<v Speaker 1>He sent no bacterium, no virus, no fungus, no worm,

0:39:23.920 --> 0:39:32.200
<v Speaker 1>nothing thing enter Carlton Gadgetsek. I want to pause here

0:39:32.360 --> 0:39:35.040
<v Speaker 1>and warn anyone who is listening that I'm going to

0:39:35.120 --> 0:39:39.200
<v Speaker 1>talk a bit about Gadgessec's criminal history, which includes his

0:39:39.320 --> 0:39:42.879
<v Speaker 1>conviction as a child molester. So if you don't want

0:39:42.880 --> 0:39:45.200
<v Speaker 1>to hear about that, fast forward about two and a

0:39:45.239 --> 0:39:48.480
<v Speaker 1>half minutes. Let me give you a little bit of

0:39:48.480 --> 0:39:52.680
<v Speaker 1>background on this piece of work. Gadgasek was a convicted

0:39:52.760 --> 0:39:56.480
<v Speaker 1>child molester and self proclaimed pedophile. He was also a

0:39:56.480 --> 0:39:58.200
<v Speaker 1>Nobel Prize winning pediatrician.

0:39:58.800 --> 0:40:00.479
<v Speaker 2>I'm sorry what?

0:40:01.160 --> 0:40:10.360
<v Speaker 1>Oh yeah, yep. Medically, Gadgisik was fascinated by rare, incurable diseases. Personally,

0:40:10.400 --> 0:40:15.080
<v Speaker 1>he was interested in so called non Western sexuality, particularly

0:40:15.120 --> 0:40:16.320
<v Speaker 1>as it related to children.

0:40:17.080 --> 0:40:19.240
<v Speaker 2>Ew I hate this already.

0:40:19.440 --> 0:40:23.799
<v Speaker 1>Yeah, he's a despicable human being. He was not well

0:40:23.920 --> 0:40:27.120
<v Speaker 1>liked by many of his colleagues, or liked at all,

0:40:27.640 --> 0:40:31.279
<v Speaker 1>and some would refer to him as inhuman and just

0:40:31.320 --> 0:40:34.920
<v Speaker 1>short of a sociopath. Along the path of his research

0:40:34.960 --> 0:40:38.960
<v Speaker 1>were scattered the remains of former collaborations and friendships. At

0:40:38.960 --> 0:40:42.880
<v Speaker 1>the same time, Gadgisik was widely admired by his academic

0:40:42.920 --> 0:40:48.520
<v Speaker 1>peers for his drive, ambition, and intelligence. And many of

0:40:48.520 --> 0:40:51.239
<v Speaker 1>these peers would later beg leniency from the judge for

0:40:51.280 --> 0:40:56.760
<v Speaker 1>Gadgetiic sentencing for child molestation. Quote, Carlton was a complicated man,

0:40:57.120 --> 0:41:00.920
<v Speaker 1>his peers would say, or something along the lines of of, no,

0:41:01.040 --> 0:41:04.120
<v Speaker 1>he wasn't perfect, but he was a genius, which seemed

0:41:04.160 --> 0:41:07.279
<v Speaker 1>to excuse his behavior as a child molester as the

0:41:07.280 --> 0:41:09.720
<v Speaker 1>cost of conducting important medical research.

0:41:09.960 --> 0:41:10.880
<v Speaker 2>Oh my god.

0:41:11.400 --> 0:41:14.719
<v Speaker 1>Or they would take a stance of cultural relativism. If

0:41:14.800 --> 0:41:17.839
<v Speaker 1>pedophilia was practiced in a certain culture, then it was okay.

0:41:17.880 --> 0:41:23.520
<v Speaker 1>If Gadgaseek sought out those cultures as his hunting ground. Ew, yeah,

0:41:23.680 --> 0:41:28.320
<v Speaker 1>they would they that was part of his defense. Oh.

0:41:28.640 --> 0:41:31.479
<v Speaker 1>The reason that I'm spending so much time talking about

0:41:31.520 --> 0:41:36.400
<v Speaker 1>who Gadgetick was is because I am extremely frustrated with

0:41:36.440 --> 0:41:41.000
<v Speaker 1>the fact that his molestations, his crimes, are so often

0:41:41.080 --> 0:41:46.160
<v Speaker 1>relegated to a footnote when discussing him or his research. Yeah,

0:41:46.600 --> 0:41:50.320
<v Speaker 1>it's so frustrating to me when I read an entire

0:41:50.440 --> 0:41:53.799
<v Speaker 1>book about preons, The Family who Couldn't Sleep discusses it

0:41:53.880 --> 0:41:58.839
<v Speaker 1>in depth, but Stanley Prisoner's book very briefly mentions this,

0:41:59.480 --> 0:42:02.880
<v Speaker 1>and it is so appalling because.

0:42:02.640 --> 0:42:05.319
<v Speaker 2>Then it just like it almost excuses it, like we

0:42:05.320 --> 0:42:08.160
<v Speaker 2>can just ignore that piece of history exactly.

0:42:09.000 --> 0:42:11.719
<v Speaker 1>So now that you know who Gadgisick was, let's get

0:42:11.760 --> 0:42:13.680
<v Speaker 1>back to Papua and New Guinea. In the nineteen fifties,

0:42:14.719 --> 0:42:17.799
<v Speaker 1>Gadgisick was always on the hunt for new diseases and new,

0:42:17.880 --> 0:42:22.200
<v Speaker 1>in his words, uncivilized places to explore. When he was

0:42:22.239 --> 0:42:24.879
<v Speaker 1>in Australia looking for his next adventure, he learned about

0:42:24.880 --> 0:42:28.600
<v Speaker 1>this mysterious illness that was devastating the foray this could

0:42:28.600 --> 0:42:30.800
<v Speaker 1>be the medical find of the century, and he wanted

0:42:30.840 --> 0:42:32.960
<v Speaker 1>to be a part of it, not just a part

0:42:32.960 --> 0:42:36.000
<v Speaker 1>of it, the whole of it. He shouldered his way

0:42:36.200 --> 0:42:39.680
<v Speaker 1>into the investigation and took it over, irritating many people

0:42:39.719 --> 0:42:42.439
<v Speaker 1>along the way who started to wonder if he had

0:42:42.440 --> 0:42:43.560
<v Speaker 1>a medical degree at all.

0:42:44.120 --> 0:42:44.400
<v Speaker 2>Wow.

0:42:44.960 --> 0:42:48.960
<v Speaker 1>Yeah. Once in Papua New Guinea, he took samples and

0:42:49.040 --> 0:42:53.040
<v Speaker 1>more samples and tracked cases as the numbers climbed every month.

0:42:53.640 --> 0:42:56.160
<v Speaker 1>In June of nineteen forty seven, he had recorded over

0:42:56.239 --> 0:42:59.959
<v Speaker 1>two hundred deaths, and these deaths followed a strange pasath.

0:43:00.920 --> 0:43:03.920
<v Speaker 1>It appeared that for every one male struck by KUU,

0:43:04.480 --> 0:43:08.080
<v Speaker 1>up to fourteen females were sick with the disease, and

0:43:08.160 --> 0:43:11.399
<v Speaker 1>still the origin of this disease, or even its pathology,

0:43:11.440 --> 0:43:14.720
<v Speaker 1>had yet to be figured out. But slowly the pieces

0:43:14.719 --> 0:43:19.279
<v Speaker 1>were falling into place. First, a researcher working in one

0:43:19.320 --> 0:43:22.080
<v Speaker 1>of the labs to which Gadgasik had sent victims' brains

0:43:22.719 --> 0:43:26.440
<v Speaker 1>noticed strange deformities in some nerve cells, and these deformities

0:43:26.480 --> 0:43:28.759
<v Speaker 1>reminded him of a disease he had just seen in

0:43:28.800 --> 0:43:31.280
<v Speaker 1>a textbook, Krutzfeldt Yakub disease.

0:43:32.040 --> 0:43:33.319
<v Speaker 2>Oh yeah.

0:43:35.120 --> 0:43:37.680
<v Speaker 1>Anxious to make similar headway on the source of the disease,

0:43:38.160 --> 0:43:41.640
<v Speaker 1>gadgasek supervisor told him he wanted to assemble a team

0:43:41.680 --> 0:43:48.480
<v Speaker 1>of anthropologists, epidemiologist, physicians, et cetera. But Gadgasik refused. Couldn't

0:43:48.480 --> 0:43:51.320
<v Speaker 1>they understand he was the team?

0:43:51.680 --> 0:43:52.320
<v Speaker 2>Oh my god.

0:43:52.760 --> 0:43:58.400
<v Speaker 1>Yeah, But he wouldn't end up actually solving that riddle,

0:43:58.800 --> 0:44:00.919
<v Speaker 1>even though he would later claim to have known about

0:44:00.920 --> 0:44:02.600
<v Speaker 1>the disease's origin all along.

0:44:03.239 --> 0:44:05.640
<v Speaker 2>Oh cool, Yeah, No, I knew about it. I just

0:44:05.680 --> 0:44:06.600
<v Speaker 2>didn't tell you guys.

0:44:06.920 --> 0:44:09.720
<v Speaker 1>Yeah, he said it was too obvious to publish.

0:44:10.040 --> 0:44:16.080
<v Speaker 2>Oh my god. Yeah, I don't know that I've ever

0:44:16.120 --> 0:44:19.120
<v Speaker 2>hated a person that we've talked about on this podcast more.

0:44:20.440 --> 0:44:24.520
<v Speaker 1>I despise him. No, the origin of the disease would

0:44:24.600 --> 0:44:30.520
<v Speaker 1>actually be uncovered by anthropologist Shirley Lindenbaum and medical researcher

0:44:30.719 --> 0:44:32.000
<v Speaker 1>Michael Alpers.

0:44:32.440 --> 0:44:36.080
<v Speaker 2>Yeah, Sureley and Michael too.

0:44:37.239 --> 0:44:40.640
<v Speaker 1>They did the careful, methodical work that Gadgasek never had

0:44:40.640 --> 0:44:44.760
<v Speaker 1>the time for, like drawing family trees and tracing relationships,

0:44:45.160 --> 0:44:49.640
<v Speaker 1>and more importantly, they listened to the Foray. Since the

0:44:49.719 --> 0:44:54.040
<v Speaker 1>disease was relatively new to the Foray, dating back only

0:44:54.080 --> 0:44:57.000
<v Speaker 1>forty or fifty years, whatever had caused it was probably

0:44:57.040 --> 0:45:00.200
<v Speaker 1>new as well. And while numerous people had noted their

0:45:00.200 --> 0:45:04.520
<v Speaker 1>observations of cannibalism practiced by the Fora, what they didn't

0:45:04.560 --> 0:45:07.680
<v Speaker 1>know was what these two researchers would learn by listening.

0:45:08.520 --> 0:45:12.160
<v Speaker 1>Cannibalism had only been adopted by the Forae about fifty

0:45:12.280 --> 0:45:20.600
<v Speaker 1>years prior Aha Yeah, timelines line enough. This wasn't revenge

0:45:20.640 --> 0:45:24.920
<v Speaker 1>cannibalism eating their enemies. The Fora used cannibalism to honor

0:45:24.960 --> 0:45:28.560
<v Speaker 1>and mourn their dead loved ones, and this practice seemed

0:45:28.600 --> 0:45:31.239
<v Speaker 1>to be at the root of the Kuru epidemic. It

0:45:31.320 --> 0:45:34.160
<v Speaker 1>explained the recent emergence of the disease and the skewed

0:45:34.200 --> 0:45:38.239
<v Speaker 1>sex ratio, and when the Forae stopped practicing cannibalism at

0:45:38.280 --> 0:45:43.000
<v Speaker 1>the insistence of some missionaries, the disease started its decline.

0:45:43.160 --> 0:45:48.520
<v Speaker 1>This was a controversial explanation for many reasons. But if

0:45:48.560 --> 0:45:51.680
<v Speaker 1>this was an infectious disease, it was unlike any that

0:45:51.760 --> 0:45:56.480
<v Speaker 1>had been described. The years long, decades long incubation period.

0:45:57.239 --> 0:46:01.560
<v Speaker 1>The absence of any detectable virus or bacterium, it was bizarre.

0:46:02.280 --> 0:46:05.399
<v Speaker 1>Around nineteen sixty, Gadgasek returned to the US to work

0:46:05.440 --> 0:46:08.360
<v Speaker 1>on Kuru in a lab setting when he was contacted

0:46:08.360 --> 0:46:12.080
<v Speaker 1>by a Scrapie researcher named William Hadlow, who had seen

0:46:12.120 --> 0:46:16.120
<v Speaker 1>an exhibit on Kuru in a museum that included pictures

0:46:16.200 --> 0:46:21.440
<v Speaker 1>of brain tissue showing the damage. Oh Hadlow thought that

0:46:21.560 --> 0:46:25.480
<v Speaker 1>Kuru looked strikingly similar to Scrapie, so he published this

0:46:25.520 --> 0:46:31.120
<v Speaker 1>observation and also wrote to Gadgetsek directly earlier. I left

0:46:31.120 --> 0:46:33.799
<v Speaker 1>off with Scrapie in the early eighteen hundreds, but the

0:46:33.840 --> 0:46:38.120
<v Speaker 1>disease hadn't disappeared. Occasional outbreak still occurred in sheep all

0:46:38.160 --> 0:46:40.759
<v Speaker 1>over Europe, and it showed up an American sheep in

0:46:40.840 --> 0:46:43.520
<v Speaker 1>nineteen forty seven, so it was still getting a lot

0:46:43.520 --> 0:46:47.600
<v Speaker 1>of research attention. The biggest development with Scrapye, though, came

0:46:47.640 --> 0:46:51.040
<v Speaker 1>about when it was shown to be transmissible after thousands

0:46:51.080 --> 0:46:55.000
<v Speaker 1>of sheep developed the disease after being given a vaccine

0:46:55.080 --> 0:46:59.839
<v Speaker 1>for looping ill. For what looping ill? It's a tick

0:47:00.080 --> 0:47:00.520
<v Speaker 1>or illness?

0:47:02.200 --> 0:47:03.439
<v Speaker 2>What a ridiculous name.

0:47:03.760 --> 0:47:08.160
<v Speaker 1>We'll cover it, We'll cover someday. Good. The vaccine had

0:47:08.160 --> 0:47:11.480
<v Speaker 1>been prepared from sheep brains that had been treated with formulin,

0:47:12.080 --> 0:47:16.040
<v Speaker 1>which should kill pretty much all living things. It sure should,

0:47:16.640 --> 0:47:18.680
<v Speaker 1>but not Preon's okay.

0:47:18.719 --> 0:47:21.279
<v Speaker 2>Also, people don't get scared of vaccines because of this,

0:47:21.360 --> 0:47:25.359
<v Speaker 2>because we don't make vaccines from brains for humans. Okay, now,

0:47:25.400 --> 0:47:26.760
<v Speaker 2>we don't relax.

0:47:28.960 --> 0:47:31.239
<v Speaker 1>This also showed that there could be a very long

0:47:31.280 --> 0:47:34.680
<v Speaker 1>period from exposure to development of symptoms, just like Kuru.

0:47:35.719 --> 0:47:38.719
<v Speaker 1>The note from Hadlow gave Gadgetsek the idea to see

0:47:38.719 --> 0:47:43.240
<v Speaker 1>whether Kuru could also be transmitted. He set about acquiring chimpanzees,

0:47:43.480 --> 0:47:46.840
<v Speaker 1>other primates, mice, hamsters, basically whatever he could get his

0:47:46.920 --> 0:47:50.719
<v Speaker 1>hands on to inject with tissue from Kuru victims. He

0:47:50.840 --> 0:47:53.320
<v Speaker 1>left someone else in charge of doing the actual work

0:47:53.800 --> 0:47:59.920
<v Speaker 1>and went to go explore new places. Within twenty one months,

0:48:00.239 --> 0:48:03.719
<v Speaker 1>which is an eternity. In the lab, a chimpanzee named

0:48:03.719 --> 0:48:07.360
<v Speaker 1>Georgette started showing signs of the disease. Wow.

0:48:07.960 --> 0:48:10.239
<v Speaker 2>I never thought about how difficult it would be to

0:48:10.280 --> 0:48:13.319
<v Speaker 2>do research on Preon's in the lab because of how

0:48:13.320 --> 0:48:14.680
<v Speaker 2>long the incubation period is.

0:48:15.080 --> 0:48:19.640
<v Speaker 1>It's really difficult, and I think very emotionally taxing.

0:48:20.080 --> 0:48:20.319
<v Speaker 3>Yeah.

0:48:20.360 --> 0:48:24.520
<v Speaker 2>Also, it's a gamble, right, yeah, ma'am.

0:48:25.200 --> 0:48:27.360
<v Speaker 1>One by one the rest of the chimps developed what

0:48:27.440 --> 0:48:32.719
<v Speaker 1>looked like Kuru and died. Oh poor babies, I know. Georgette.

0:48:34.000 --> 0:48:36.560
<v Speaker 1>The damage to their brains looked incredibly similar to that

0:48:36.640 --> 0:48:40.799
<v Speaker 1>of Kuru scrapy and kruz felt yakub sufferers and Gayjsek

0:48:40.880 --> 0:48:44.080
<v Speaker 1>ordered more injections, not just with Kuru, but with tissue

0:48:44.120 --> 0:48:50.320
<v Speaker 1>from other neurodegenerative diseases as well. These experiments show that Kuru, scrapy,

0:48:50.360 --> 0:48:54.279
<v Speaker 1>and kruzfeld yaka could all be transmitted. He published his

0:48:54.400 --> 0:48:57.920
<v Speaker 1>results and hypothesized that these three diseases were all caused

0:48:57.920 --> 0:49:02.000
<v Speaker 1>by the same thing, a slow vibe, a vague explanation

0:49:02.080 --> 0:49:06.759
<v Speaker 1>that he didn't really expand on. For this and for

0:49:06.840 --> 0:49:09.240
<v Speaker 1>his description of Kuru, he would be awarded the Nobel

0:49:09.320 --> 0:49:14.000
<v Speaker 1>Prize in nineteen seventy six. Jeez, yeah, a decision that

0:49:14.040 --> 0:49:16.400
<v Speaker 1>baffled many, including me.

0:49:17.360 --> 0:49:18.480
<v Speaker 2>Can they take it away?

0:49:18.840 --> 0:49:18.920
<v Speaker 1>Like?

0:49:20.200 --> 0:49:22.080
<v Speaker 2>Does that ever get taken away?

0:49:22.480 --> 0:49:26.000
<v Speaker 1>That's a great question. I don't know. We should look

0:49:26.000 --> 0:49:26.359
<v Speaker 1>into that.

0:49:26.560 --> 0:49:27.520
<v Speaker 2>We should look into it.

0:49:29.280 --> 0:49:31.960
<v Speaker 1>Meanwhile, plenty of other researchers were hard at work on

0:49:32.040 --> 0:49:36.279
<v Speaker 1>these diseases, such as Tikva Alper, who demonstrated that even

0:49:36.400 --> 0:49:42.000
<v Speaker 1>radiation could not destroy the scrapy particle. WHA. I also

0:49:42.200 --> 0:49:46.680
<v Speaker 1>have to say she sounds amazing. So she refused to

0:49:46.800 --> 0:49:50.360
<v Speaker 1>accept a royal sixtieth wedding anniversary greeting from the Queen

0:49:50.800 --> 0:49:53.760
<v Speaker 1>because it was addressed to her and her husband under

0:49:53.840 --> 0:49:56.560
<v Speaker 1>his name, even though she didn't take his last name.

0:49:56.840 --> 0:50:00.920
<v Speaker 2>Oh my god, she is. I I feel so strongly

0:50:00.920 --> 0:50:03.879
<v Speaker 2>about Whenever we get a letter that's addressed to mister

0:50:04.000 --> 0:50:07.080
<v Speaker 2>and missus Brett Updike, I throw it at him and

0:50:07.120 --> 0:50:10.759
<v Speaker 2>I'm like, this is for you, which like it's not

0:50:10.840 --> 0:50:16.400
<v Speaker 2>his fault, but oh my god, I feel that so strongly.

0:50:16.800 --> 0:50:20.960
<v Speaker 1>She's awesome. Another big discovery was that there seemed to

0:50:20.960 --> 0:50:24.400
<v Speaker 1>be multiple strains of scrapie, judging by the different patterns

0:50:24.400 --> 0:50:28.880
<v Speaker 1>of disease. But still, what was the agent? Where was

0:50:28.920 --> 0:50:33.680
<v Speaker 1>the virus? How could it survive being irradiated, desiccated, cooked.

0:50:34.840 --> 0:50:38.400
<v Speaker 1>Researchers could isolate the protein coat of this mysterious virus

0:50:38.400 --> 0:50:42.320
<v Speaker 1>that supposedly existed, but they couldn't detect any nucleic acids.

0:50:42.760 --> 0:50:44.360
<v Speaker 1>I'm getting so excited.

0:50:45.400 --> 0:50:47.440
<v Speaker 2>I'm sorry, I'm getting so excited.

0:50:48.280 --> 0:50:52.040
<v Speaker 1>Could it actually be just a protein causing these diseases?

0:50:52.600 --> 0:50:53.040
<v Speaker 2>Couldn't?

0:50:54.239 --> 0:50:58.759
<v Speaker 1>Most researchers gave a firm no, but a handful we're

0:50:58.760 --> 0:51:02.759
<v Speaker 1>more open minded and largely ignored Tikva Albert was in

0:51:02.840 --> 0:51:06.799
<v Speaker 1>this open minded and largely ignored capillar. By way, a

0:51:06.840 --> 0:51:11.800
<v Speaker 1>British mathematician actually named J. S. Griffith publishes a paper

0:51:11.920 --> 0:51:15.400
<v Speaker 1>proposing several different mechanisms by which it could be a protein.

0:51:15.880 --> 0:51:18.480
<v Speaker 1>But it doesn't really gain a lot of traction and

0:51:18.960 --> 0:51:23.440
<v Speaker 1>the scrapy Koru krusfeldt jakub slow virus field was about

0:51:23.440 --> 0:51:26.480
<v Speaker 1>to gain a new member who would give prions their

0:51:26.560 --> 0:51:32.120
<v Speaker 1>name and earn a little notoriety for himself. Meet Stanley Prisoner, MD,

0:51:35.880 --> 0:51:39.960
<v Speaker 1>with a background in neurology and biochemistry. Prisoner's career trajectory

0:51:40.040 --> 0:51:42.480
<v Speaker 1>became a whole lot more focused when he saw his

0:51:42.520 --> 0:51:46.799
<v Speaker 1>first Chrisfeldt Yakab patient in nineteen seventy two. This was

0:51:46.800 --> 0:51:49.840
<v Speaker 1>a problem he knew he wanted to work on. He

0:51:49.920 --> 0:51:53.840
<v Speaker 1>collaborated with the scrapy researcher William Hadlow and began attempting

0:51:53.880 --> 0:51:58.239
<v Speaker 1>to purify the scrapy particle. Having that isolated agent was

0:51:58.360 --> 0:52:01.560
<v Speaker 1>super important because you could do experiments directly on it

0:52:01.600 --> 0:52:04.799
<v Speaker 1>to see how it would react. For instance, Prisoner and

0:52:04.840 --> 0:52:07.359
<v Speaker 1>his group showed that the particle did not lose its

0:52:07.400 --> 0:52:11.920
<v Speaker 1>infectivity when treated with chemicals that destroyed nucleic acids, but

0:52:12.600 --> 0:52:15.560
<v Speaker 1>it did lose its infectivity when it was treated with

0:52:15.680 --> 0:52:18.359
<v Speaker 1>chemicals that destroyed proteins.

0:52:18.800 --> 0:52:20.360
<v Speaker 2>Oh boom, boom boom.

0:52:22.000 --> 0:52:25.400
<v Speaker 1>With this purified particle, you could also design an antibody

0:52:25.400 --> 0:52:29.759
<v Speaker 1>test for it, which really spread up diagnosis. Previously, you

0:52:29.800 --> 0:52:32.880
<v Speaker 1>would have had to inject a lab animal with tissue

0:52:33.360 --> 0:52:36.160
<v Speaker 1>from a person or sheep or whatever, and then wait

0:52:36.239 --> 0:52:39.160
<v Speaker 1>for signs of disease for conformation, which can take at

0:52:39.200 --> 0:52:43.359
<v Speaker 1>least a year. Yeah, the field of preon research, as

0:52:43.400 --> 0:52:46.200
<v Speaker 1>it would soon be known, seems to have been filled

0:52:46.600 --> 0:52:51.800
<v Speaker 1>with egos, with Prisoners perhaps the largest.

0:52:52.000 --> 0:52:53.080
<v Speaker 2>Wow I'm shocked.

0:52:53.560 --> 0:52:56.560
<v Speaker 1>Yeah, to say that he was not well liked by

0:52:56.600 --> 0:53:01.719
<v Speaker 1>most of the field is probably understating it. Prisoner was

0:53:01.840 --> 0:53:06.719
<v Speaker 1>fiercely driven, often combative, selfish with his findings, and obsessed

0:53:06.719 --> 0:53:07.520
<v Speaker 1>with getting credit.

0:53:08.120 --> 0:53:08.640
<v Speaker 2>God.

0:53:09.120 --> 0:53:12.080
<v Speaker 1>He painted himself as a martyr and fighting the good

0:53:12.080 --> 0:53:15.560
<v Speaker 1>fight against conventional thought. And part of the reason that

0:53:15.600 --> 0:53:18.360
<v Speaker 1>I say this is because it was how he was

0:53:18.400 --> 0:53:21.600
<v Speaker 1>written about in the Preon book that I read, which

0:53:21.680 --> 0:53:24.680
<v Speaker 1>I understand is going to be It's a story, right,

0:53:24.719 --> 0:53:28.319
<v Speaker 1>it comes from a certain angles biased. But I also

0:53:28.480 --> 0:53:33.040
<v Speaker 1>read the book that he wrote, his memoir, and this

0:53:33.120 --> 0:53:39.040
<v Speaker 1>comes through very strongly in that as well. Oh yeah, Anyway,

0:53:39.280 --> 0:53:43.160
<v Speaker 1>Prisoner really did advance this field, and one way he

0:53:43.200 --> 0:53:45.400
<v Speaker 1>did that was to give it its name. When he

0:53:45.440 --> 0:53:48.319
<v Speaker 1>started working in this area, Kuru, Scrapie and Chris felt

0:53:48.360 --> 0:53:51.479
<v Speaker 1>Yaka were all still generally referred to by the term

0:53:51.640 --> 0:53:55.920
<v Speaker 1>given to it by Gadgetsek slow virus. Prisoner didn't like

0:53:56.040 --> 0:53:58.839
<v Speaker 1>this name. It wasn't really accurate since no virus had

0:53:58.880 --> 0:54:01.400
<v Speaker 1>been found, so he set about trying to think of

0:54:01.440 --> 0:54:04.640
<v Speaker 1>a new one. He came up with Preon for a

0:54:04.640 --> 0:54:11.480
<v Speaker 1>proteinaceous infectious particle, so creative props. This wasn't a popular

0:54:11.520 --> 0:54:15.279
<v Speaker 1>move in the field. First of all, he was criticized

0:54:15.280 --> 0:54:19.719
<v Speaker 1>for making it sound like his own name. Secondly, it

0:54:19.840 --> 0:54:23.560
<v Speaker 1>dodged the question of what this particle actually was. Was

0:54:23.600 --> 0:54:28.440
<v Speaker 1>a preon, a protein, a virus, or just a particle?

0:54:32.320 --> 0:54:34.600
<v Speaker 2>Just a particle, man, Relax.

0:54:36.800 --> 0:54:39.800
<v Speaker 1>Despite the disdain for this word, it worked, The press

0:54:39.840 --> 0:54:42.400
<v Speaker 1>loved it, and it rolled right off the tongue. But

0:54:42.480 --> 0:54:45.080
<v Speaker 1>prisoners still needed to get more evidence for the protein

0:54:45.120 --> 0:54:49.480
<v Speaker 1>only hypothesis. To show that a protein could be infectious,

0:54:50.040 --> 0:54:53.359
<v Speaker 1>he had to synthesize a protein and then introduce it

0:54:53.360 --> 0:54:54.040
<v Speaker 1>to an animal.

0:54:54.480 --> 0:54:56.880
<v Speaker 2>I'm so excited about this part because I read a

0:54:56.920 --> 0:54:59.520
<v Speaker 2>little bit about it in the biology and I can't

0:54:59.520 --> 0:55:00.719
<v Speaker 2>wait to hear more about it.

0:55:00.800 --> 0:55:03.279
<v Speaker 1>Oh Man. I hope that I give you enough of

0:55:03.280 --> 0:55:07.200
<v Speaker 1>what you're looking for. I'm not sure. So. A problem

0:55:07.239 --> 0:55:11.360
<v Speaker 1>arose when they had purified this protein only to find

0:55:11.360 --> 0:55:15.120
<v Speaker 1>out that it was just an ordinary protein produced by

0:55:15.120 --> 0:55:15.840
<v Speaker 1>the own host.

0:55:17.560 --> 0:55:21.759
<v Speaker 2>Can you imagine how bizarre that must have been. You're like, no,

0:55:21.800 --> 0:55:24.640
<v Speaker 2>we really think we've got it. It's this thing what.

0:55:25.280 --> 0:55:29.040
<v Speaker 1>Yeah. It called into question the entire concept of an

0:55:29.080 --> 0:55:35.080
<v Speaker 1>infectious protein, oh Man, especially when he in collaboration with

0:55:35.120 --> 0:55:39.200
<v Speaker 1>other labs found the location of this gene that produced

0:55:39.200 --> 0:55:42.040
<v Speaker 1>the preon. Yeah, and he found that it was highly

0:55:42.080 --> 0:55:45.640
<v Speaker 1>conserved across so many animal species, indicating that it was

0:55:45.680 --> 0:55:49.000
<v Speaker 1>probably super important to keep right.

0:55:48.880 --> 0:55:51.239
<v Speaker 2>It's this gene that like all these mammals have. That's

0:55:51.320 --> 0:55:54.520
<v Speaker 2>why you get preon diseases in so many different mammals.

0:55:54.800 --> 0:55:56.960
<v Speaker 1>Yeah, it must have just been like, well, this was

0:55:57.000 --> 0:56:00.279
<v Speaker 1>a wild use chase. How are we so wrong? Right?

0:56:00.320 --> 0:56:01.480
<v Speaker 2>Where do we go from here?

0:56:01.840 --> 0:56:02.520
<v Speaker 1>Where did they go?

0:56:02.840 --> 0:56:03.200
<v Speaker 2>Aaron?

0:56:05.120 --> 0:56:07.640
<v Speaker 1>Okay, So, if the only function of this gene was

0:56:07.680 --> 0:56:10.879
<v Speaker 1>to produce a protein that killed the animal, why would

0:56:10.880 --> 0:56:14.200
<v Speaker 1>it still be around? How could it still be around?

0:56:14.280 --> 0:56:17.440
<v Speaker 1>Prisoner had an answer for that too, the same one

0:56:17.480 --> 0:56:21.520
<v Speaker 1>that the mathematician Griffith had proposed years earlier. A protein

0:56:21.600 --> 0:56:24.279
<v Speaker 1>could have two forms, one that was normal and the

0:56:24.320 --> 0:56:25.760
<v Speaker 1>other that was disease causing.

0:56:26.560 --> 0:56:31.759
<v Speaker 2>I'm sorry, this is the first time that people realized

0:56:31.760 --> 0:56:35.120
<v Speaker 2>that proteins could misfold was because of preons.

0:56:35.880 --> 0:56:40.279
<v Speaker 1>I don't know, but that is so cool. Yeah, I

0:56:40.320 --> 0:56:43.480
<v Speaker 1>think so. I think it is because at this point

0:56:43.520 --> 0:56:46.560
<v Speaker 1>this was still just a concept. It had not been

0:56:46.640 --> 0:56:51.520
<v Speaker 1>observed or demonstrated that proteins could do this. Oh, my gosh,

0:56:51.920 --> 0:56:55.760
<v Speaker 1>there needed to be an experiment. Yeah, and there actually

0:56:55.920 --> 0:57:00.960
<v Speaker 1>kind of was one that nature had already set up. So,

0:57:01.880 --> 0:57:05.600
<v Speaker 1>like you talked about, some prion diseases can be inherited,

0:57:05.920 --> 0:57:10.640
<v Speaker 1>like khruslat Yaka disease and GSS, and those diseases show

0:57:10.680 --> 0:57:13.640
<v Speaker 1>that there were mutations in the prion gene leading to

0:57:13.680 --> 0:57:18.120
<v Speaker 1>a state of disease. So prisoner created mice with mutations

0:57:18.160 --> 0:57:21.520
<v Speaker 1>in this gene. He observed them getting sick, killed them,

0:57:22.000 --> 0:57:25.760
<v Speaker 1>and then took the infectious prions from them and injected

0:57:25.800 --> 0:57:30.240
<v Speaker 1>them into mice that didn't have the mutation and those

0:57:30.280 --> 0:57:35.720
<v Speaker 1>got sick. And yeah, so you could see easily how

0:57:35.720 --> 0:57:38.320
<v Speaker 1>a protein could be normal or infectious.

0:57:38.600 --> 0:57:42.000
<v Speaker 2>Oh, my gracious.

0:57:42.600 --> 0:57:47.680
<v Speaker 1>But but there still remained the mystery of the sporadic case.

0:57:48.240 --> 0:57:48.680
<v Speaker 2>Yeah.

0:57:49.120 --> 0:57:53.560
<v Speaker 1>So krusladyaka, like you said, could be infectious as had

0:57:53.560 --> 0:57:56.880
<v Speaker 1>been demonstrated, it could be inherited as it often was,

0:57:57.480 --> 0:58:01.760
<v Speaker 1>but sometimes it would just appear randomly. Yeah, this sporadic

0:58:01.880 --> 0:58:04.520
<v Speaker 1>form of the disease was a bit harder to explain.

0:58:05.360 --> 0:58:08.400
<v Speaker 1>If someone had a normal, non disease causing form of

0:58:08.440 --> 0:58:13.040
<v Speaker 1>the preon gene, how could the disease suddenly develop prisoner

0:58:13.080 --> 0:58:18.360
<v Speaker 1>once again had an answer, conformational influence. This doesn't fully

0:58:18.400 --> 0:58:22.280
<v Speaker 1>explain it, really, but this is basically the concept of

0:58:22.320 --> 0:58:25.960
<v Speaker 1>the domino effect of one misfolded protein causing all the

0:58:26.040 --> 0:58:29.000
<v Speaker 1>other ones that came into contact with to readjust themselves.

0:58:29.920 --> 0:58:36.720
<v Speaker 1>But this completed the three components of infectious, familial, and

0:58:37.200 --> 0:58:42.360
<v Speaker 1>sporadic to create this unified preon protein only hypothesis.

0:58:43.640 --> 0:58:46.480
<v Speaker 2>I mean, it still doesn't explain how the first one

0:58:46.520 --> 0:58:49.240
<v Speaker 2>becomes misfolded sporadically in a.

0:58:49.200 --> 0:58:52.920
<v Speaker 1>Person unless it's just probability.

0:58:52.640 --> 0:58:56.160
<v Speaker 2>Right, exactly, Yeah, because then it's just if just randomly

0:58:56.360 --> 0:58:57.600
<v Speaker 2>it might get misfolded.

0:58:58.360 --> 0:58:59.000
<v Speaker 3>Yeah.

0:58:59.200 --> 0:59:04.640
<v Speaker 1>But oh man, it's yeah. I know that we keep

0:59:04.680 --> 0:59:11.560
<v Speaker 1>saying this, but prions are super bizarre, and it's it's

0:59:11.640 --> 0:59:14.600
<v Speaker 1>no surprise that they remained such a mystery for so long,

0:59:15.120 --> 0:59:17.920
<v Speaker 1>and it's really it's more incredible that some of their

0:59:17.960 --> 0:59:22.640
<v Speaker 1>secrets have been revealed at all. I fee, yeah, I'm

0:59:22.680 --> 0:59:26.360
<v Speaker 1>also presenting this research as though it was accepted as

0:59:26.400 --> 0:59:29.760
<v Speaker 1>a fact as it happened, but in reality, it was

0:59:29.840 --> 0:59:33.840
<v Speaker 1>and continues to be debated. There are still researchers who

0:59:33.880 --> 0:59:36.439
<v Speaker 1>firmly believe that there is a tiny virus hiding out

0:59:36.480 --> 0:59:40.320
<v Speaker 1>that will one day be uncovered. Yeah, but Prisoner was

0:59:40.360 --> 0:59:44.440
<v Speaker 1>awarded the Nobel Prize for a new biological principle of

0:59:44.440 --> 0:59:49.600
<v Speaker 1>infection in nineteen ninety seven. When you hear the word prion,

0:59:50.880 --> 0:59:53.280
<v Speaker 1>what's the first disease that comes to your mind?

0:59:53.600 --> 0:59:54.120
<v Speaker 2>Mad cow?

0:59:55.560 --> 1:00:01.640
<v Speaker 1>Of course, of course it is. Mad cow disease reared

1:00:01.680 --> 1:00:04.400
<v Speaker 1>its ugly head in the late nineteen eighties in Britain

1:00:04.760 --> 1:00:08.920
<v Speaker 1>and flashed across headlines for years. It awakened the public

1:00:08.920 --> 1:00:12.880
<v Speaker 1>to tragic failures in agricultural and public health oversight. It

1:00:12.960 --> 1:00:15.800
<v Speaker 1>resulted in the deaths of hundreds of people and almost

1:00:15.880 --> 1:00:19.240
<v Speaker 1>a million cattle, and it showed how much we still

1:00:19.360 --> 1:00:23.520
<v Speaker 1>don't know about preons. It started, at least in the

1:00:23.600 --> 1:00:28.520
<v Speaker 1>nineteen seventies. Normally docile cows started acting aggressively and had

1:00:28.680 --> 1:00:33.680
<v Speaker 1>trouble walking. Death was always the outcome, Like scrapy infected sheep,

1:00:33.720 --> 1:00:36.840
<v Speaker 1>when you stroked a cow's back, it would uncontrollably nibble

1:00:36.880 --> 1:00:41.560
<v Speaker 1>and lipsmack. But the connection between scrapy and this new

1:00:41.640 --> 1:00:45.840
<v Speaker 1>disease wouldn't be made until nineteen eighty six. Wow, a

1:00:45.920 --> 1:00:48.880
<v Speaker 1>veterinary pathologist checks out some brains of a cow that

1:00:48.920 --> 1:00:52.000
<v Speaker 1>had been acting like it had scrapy. According to another vet.

1:00:52.960 --> 1:00:56.120
<v Speaker 1>Sure enough, this brain looks like scrapy in a cow,

1:00:57.000 --> 1:01:01.560
<v Speaker 1>and scrapie in a cow is huge news. Initially, the

1:01:01.600 --> 1:01:05.360
<v Speaker 1>authorities weren't that worried about the human risk. Scrapey had

1:01:05.360 --> 1:01:07.760
<v Speaker 1>never been shown to be transmitted from sheep to human,

1:01:08.560 --> 1:01:12.560
<v Speaker 1>but this might not follow that same pattern. One worrying

1:01:12.600 --> 1:01:17.120
<v Speaker 1>finding was that it had jumped species A Niala I

1:01:17.160 --> 1:01:19.360
<v Speaker 1>don't know if I'm saying that right, which is an

1:01:19.360 --> 1:01:22.560
<v Speaker 1>antelope like animal, and a local zoo had died of

1:01:22.600 --> 1:01:28.720
<v Speaker 1>a scrapy like disease. Bovine's bonge of form encephalopathy, which

1:01:28.760 --> 1:01:31.040
<v Speaker 1>is the mouthful of a name given to the disease,

1:01:31.280 --> 1:01:35.080
<v Speaker 1>started appearing all over England, and the hunt was on

1:01:35.280 --> 1:01:40.960
<v Speaker 1>for the common exposure, though quietly, of course, mustn't worry

1:01:40.960 --> 1:01:52.560
<v Speaker 1>the public, that's good. The Ministry of Agriculture, Fisheries and

1:01:52.640 --> 1:01:56.360
<v Speaker 1>Food was housed under one roof, bureaucratically speaking, and they

1:01:56.400 --> 1:01:59.680
<v Speaker 1>were in charge of both the as the name suggests,

1:02:00.160 --> 1:02:04.240
<v Speaker 1>the agricultural sector and human food safety, which kind of

1:02:04.280 --> 1:02:08.960
<v Speaker 1>seems like an inherent conflict of interest. They could either

1:02:09.080 --> 1:02:11.120
<v Speaker 1>keep the mad cow meat out of the human food

1:02:11.120 --> 1:02:15.320
<v Speaker 1>supply and economically devastate the farmers, or keep it in

1:02:15.360 --> 1:02:19.000
<v Speaker 1>and potentially expose millions of humans to an incurable disease.

1:02:20.280 --> 1:02:23.520
<v Speaker 1>The silence about the potential danger to humans didn't last long,

1:02:24.280 --> 1:02:27.920
<v Speaker 1>nor did the original name. It was soon dubbed mad

1:02:28.000 --> 1:02:31.280
<v Speaker 1>cow disease much more memorable.

1:02:31.000 --> 1:02:32.160
<v Speaker 2>So much more memorable.

1:02:33.440 --> 1:02:36.360
<v Speaker 1>At this time, mad cow meat was still making its

1:02:36.360 --> 1:02:37.960
<v Speaker 1>way into the markets.

1:02:37.760 --> 1:02:41.680
<v Speaker 2>Mad cow meat, Like, how could you call it anything

1:02:41.760 --> 1:02:43.320
<v Speaker 2>but my cow?

1:02:43.400 --> 1:02:50.040
<v Speaker 1>After that? If your cow died in the pasture, you

1:02:50.080 --> 1:02:54.000
<v Speaker 1>couldn't sell it for human consumption. But oh no, if

1:02:54.040 --> 1:02:56.960
<v Speaker 1>you got a sick cow to a slaughterhouse before it died,

1:02:57.560 --> 1:02:58.120
<v Speaker 1>that was fine.

1:02:58.200 --> 1:02:58.640
<v Speaker 2>Oh no.

1:03:02.040 --> 1:03:06.280
<v Speaker 1>Screenings found evidence of mad cow in farms all over England.

1:03:06.960 --> 1:03:11.840
<v Speaker 1>Where was it coming from? The short answer, cake in parlor?

1:03:12.320 --> 1:03:16.600
<v Speaker 1>What cake in parlor?

1:03:16.720 --> 1:03:20.240
<v Speaker 2>Like, we're having cake in the PARLYA dear? That was bad?

1:03:20.360 --> 1:03:21.440
<v Speaker 2>It precisely okay.

1:03:23.280 --> 1:03:27.360
<v Speaker 1>Cake was a high protein concentrate that farmers would give

1:03:27.360 --> 1:03:31.240
<v Speaker 1>to cows who were milking in parlor. So in parlor

1:03:31.280 --> 1:03:31.880
<v Speaker 1>means milking.

1:03:32.160 --> 1:03:36.440
<v Speaker 2>Oh, this is cows eating cake in the parlor, not humans.

1:03:36.760 --> 1:03:39.720
<v Speaker 1>Yeah, where was it coming from? In the cows got it?

1:03:42.600 --> 1:03:46.320
<v Speaker 1>The protein in this cake didn't come from soy.

1:03:46.600 --> 1:03:48.600
<v Speaker 2>Oh no, oh no.

1:03:49.160 --> 1:03:52.160
<v Speaker 1>It generally came from farm animals that couldn't be sold

1:03:52.200 --> 1:03:56.520
<v Speaker 1>to the human market. Yeah, just all bits ground up

1:03:56.560 --> 1:03:57.880
<v Speaker 1>and molded into a cake.

1:03:59.320 --> 1:04:04.040
<v Speaker 2>I'm sorry, I'm thinking about my dog food right now. Yeah,

1:04:05.920 --> 1:04:06.880
<v Speaker 2>gross man.

1:04:08.600 --> 1:04:11.200
<v Speaker 1>This explained how the disease was showing up all over

1:04:11.240 --> 1:04:13.840
<v Speaker 1>the country and how the nyala had gotten sick. The

1:04:13.920 --> 1:04:16.920
<v Speaker 1>zoo had recently switched from soy to meat pellets.

1:04:16.960 --> 1:04:20.720
<v Speaker 2>Oh my gosh, guys, cows are not carnivores.

1:04:21.640 --> 1:04:25.800
<v Speaker 1>Nope, but once they start being fed it, they they

1:04:25.880 --> 1:04:27.200
<v Speaker 1>don't do well without it.

1:04:27.240 --> 1:04:29.320
<v Speaker 2>They go mad for it.

1:04:29.960 --> 1:04:34.040
<v Speaker 1>They go mad for it. Nerd alert.

1:04:34.160 --> 1:04:35.919
<v Speaker 2>Yeah, I pushed my glasses up.

1:04:38.720 --> 1:04:42.320
<v Speaker 1>Despite this very clear evidence of how preons could be

1:04:42.360 --> 1:04:45.840
<v Speaker 1>introduced to cows through their food, it would be eight

1:04:47.000 --> 1:04:52.760
<v Speaker 1>eight whole years until animal protein and cowfeed would be banned.

1:04:54.840 --> 1:04:57.800
<v Speaker 1>The author of this book that family who Couldn't Sleep,

1:04:58.080 --> 1:05:01.280
<v Speaker 1>compared it to keeping the Broad Street water pump operating

1:05:01.360 --> 1:05:03.960
<v Speaker 1>for eight years after John Snow showed it was the

1:05:04.000 --> 1:05:04.800
<v Speaker 1>source of cholera.

1:05:05.000 --> 1:05:07.440
<v Speaker 2>We all know how bad that would have been.

1:05:08.200 --> 1:05:12.960
<v Speaker 1>Yeah, it was like, that's an analogy. I can get everyone.

1:05:13.040 --> 1:05:15.680
<v Speaker 2>Listen to episode four if you haven't already, and then

1:05:15.760 --> 1:05:16.479
<v Speaker 2>you'll get it.

1:05:16.400 --> 1:05:23.240
<v Speaker 1>Too, nice plug. Thanks. During those eight years, certain protective

1:05:23.240 --> 1:05:26.960
<v Speaker 1>measures were enacted. For instance, any cows showing signs of

1:05:27.320 --> 1:05:32.200
<v Speaker 1>bovine sponge offormencephalopathy were to be reported and killed with

1:05:32.280 --> 1:05:38.040
<v Speaker 1>full compensation. Schools banned British beef. Many people stopped eating it,

1:05:39.040 --> 1:05:43.320
<v Speaker 1>but other worrying things started to emerge. A cat nicknamed

1:05:43.520 --> 1:05:49.320
<v Speaker 1>Mad Max was diagnosed with feline sponge offormencephalopathy. I think

1:05:49.360 --> 1:05:52.880
<v Speaker 1>he was Max, and then he got named Mad Max.

1:05:53.120 --> 1:05:55.280
<v Speaker 2>That's the cutest and saddest thing.

1:05:56.120 --> 1:06:01.560
<v Speaker 1>Yeah, cows were still getting sick after Mad Kalmi was

1:06:01.640 --> 1:06:06.520
<v Speaker 1>banned from the cowfood supply. Two marmosets began showing signs

1:06:06.520 --> 1:06:10.960
<v Speaker 1>of the disease after being experimentally inoculated, demonstrating that the

1:06:11.000 --> 1:06:17.960
<v Speaker 1>disease could be spread to primates. Big news there, and finally, tragically,

1:06:18.840 --> 1:06:22.400
<v Speaker 1>human teenagers started to be diagnosed with krusfeld Yakub disease.

1:06:23.720 --> 1:06:27.040
<v Speaker 1>This was not the sporadic form. This was a new variant,

1:06:27.800 --> 1:06:32.480
<v Speaker 1>a cluster marked by a common exposure. Europe banned British beef.

1:06:33.240 --> 1:06:36.880
<v Speaker 1>Millions of cattle were slaughtered. Estimates of the number of

1:06:36.880 --> 1:06:40.240
<v Speaker 1>people who had developed this new krusfeld Yakub variant ranged

1:06:40.280 --> 1:06:44.720
<v Speaker 1>from the hundreds to the millions. As of this year

1:06:45.080 --> 1:06:48.680
<v Speaker 1>twenty eighteen, only two hundred and thirty one cases of

1:06:48.720 --> 1:06:52.960
<v Speaker 1>the disease have been reported, but screenings of tissues from

1:06:53.040 --> 1:06:56.320
<v Speaker 1>healthy people in the UK have revealed evidence of prion

1:06:56.480 --> 1:07:01.120
<v Speaker 1>infection and at least four thousand people. Wow, But it's

1:07:01.160 --> 1:07:04.200
<v Speaker 1>not really clear whether these people will eventually develop the

1:07:04.240 --> 1:07:07.440
<v Speaker 1>disease because there's still so much not known about the

1:07:07.440 --> 1:07:12.600
<v Speaker 1>tipping point from infection to disease, right, but because the

1:07:12.680 --> 1:07:17.200
<v Speaker 1>story is still unfolding. And didn't even mean to make

1:07:17.240 --> 1:07:17.560
<v Speaker 1>that pun.

1:07:17.680 --> 1:07:19.120
<v Speaker 2>That was a protein joke, guys.

1:07:20.880 --> 1:07:24.360
<v Speaker 1>And because the UK received such an enormous dose of prions,

1:07:24.520 --> 1:07:27.880
<v Speaker 1>a lot of countries have regulations against blood donations by

1:07:27.920 --> 1:07:30.840
<v Speaker 1>people who lived in the UK during the nineteen eighties

1:07:30.840 --> 1:07:35.080
<v Speaker 1>and nineteen nineties. Yeah, mad cow has also popped up

1:07:35.120 --> 1:07:39.560
<v Speaker 1>elsewhere in the world, including the US. Many mysteries remain

1:07:39.680 --> 1:07:44.040
<v Speaker 1>about the mad cow disease outbreak. For instance, why England

1:07:44.640 --> 1:07:48.800
<v Speaker 1>howard cows still getting sick? Proteins are really tough things.

1:07:48.920 --> 1:07:53.600
<v Speaker 1>Even preon ash is apparently infectious, sure, And why did

1:07:53.680 --> 1:07:57.960
<v Speaker 1>only a handful of people get sick. The answer to

1:07:57.960 --> 1:08:02.960
<v Speaker 1>that last question might be in our There is variation

1:08:03.120 --> 1:08:06.840
<v Speaker 1>and susceptibility to preon diseases not just among people, but

1:08:06.880 --> 1:08:12.360
<v Speaker 1>also among animals like sheep and scrapy resistant breeds. Molecular

1:08:12.400 --> 1:08:15.400
<v Speaker 1>research has shown that those who have two copies of

1:08:15.440 --> 1:08:18.080
<v Speaker 1>the mutated gene are more likely to get the disease,

1:08:18.680 --> 1:08:21.559
<v Speaker 1>while those who have one normal and one mutated copy

1:08:21.600 --> 1:08:24.000
<v Speaker 1>of the gene never get the disease but can act

1:08:24.000 --> 1:08:28.200
<v Speaker 1>as a carrier. Nearly all of those who developed variant

1:08:28.240 --> 1:08:31.160
<v Speaker 1>krusfeld yakub from infected beef had two copies of this

1:08:31.280 --> 1:08:35.639
<v Speaker 1>mutated gene. More recent research shows that in the four

1:08:35.680 --> 1:08:39.120
<v Speaker 1>A people, a new variation in the prion protein emerged

1:08:39.320 --> 1:08:43.880
<v Speaker 1>super recently, maybe ten generations ago, and those with this

1:08:44.000 --> 1:08:48.639
<v Speaker 1>variation have resistance to KUU. That's genetic evolution. That's happening

1:08:48.680 --> 1:08:50.639
<v Speaker 1>on a scale we can observe.

1:08:51.080 --> 1:08:51.639
<v Speaker 2>So cool.

1:08:52.680 --> 1:08:58.920
<v Speaker 1>Yeah. The final chapter almost done of preon history. I

1:08:58.960 --> 1:09:01.600
<v Speaker 1>want to cover is a short one and one that

1:09:01.640 --> 1:09:04.160
<v Speaker 1>will take us back to the present, to the forests

1:09:04.200 --> 1:09:07.160
<v Speaker 1>of North America where thousands of deer and elk and

1:09:07.200 --> 1:09:11.719
<v Speaker 1>moose are dying of chronic wasting disease, yet another preon disease.

1:09:13.080 --> 1:09:16.799
<v Speaker 1>Where did this one come from? As usual, the answer

1:09:16.880 --> 1:09:20.840
<v Speaker 1>isn't entirely clear, but we may have a guess. In

1:09:20.880 --> 1:09:25.840
<v Speaker 1>the nineteen sixties, young biologist Jeane Schoonveld decided to study

1:09:25.880 --> 1:09:30.000
<v Speaker 1>starvation and mule deer in Fort Collins, Colorado, Laura and

1:09:30.080 --> 1:09:37.080
<v Speaker 1>Rick Okay to Laura, Schoonveld kept some starving deer in

1:09:37.160 --> 1:09:39.840
<v Speaker 1>one pen and controlled deer in another pen so he

1:09:39.880 --> 1:09:43.920
<v Speaker 1>could compare the two. This control pen also happened to

1:09:44.000 --> 1:09:50.200
<v Speaker 1>house sheep. Ooh. Now, there isn't universal agreement on this,

1:09:50.640 --> 1:09:54.559
<v Speaker 1>but several people on the project say that scrapie was

1:09:54.680 --> 1:09:58.559
<v Speaker 1>present in those sheep. If that were the case, scrapie

1:09:58.600 --> 1:10:01.160
<v Speaker 1>could have passed from the sheep to the deer who

1:10:01.200 --> 1:10:05.679
<v Speaker 1>began showing signs of disease. Wow. After the experiment was over,

1:10:06.000 --> 1:10:09.880
<v Speaker 1>Schuenveld released the control deer, so the ones who had

1:10:09.920 --> 1:10:13.040
<v Speaker 1>been in the enclosures with the sheep back into the wild.

1:10:14.560 --> 1:10:18.439
<v Speaker 1>Ten years later, researchers started noticing scrapy like behavior and

1:10:18.520 --> 1:10:22.920
<v Speaker 1>brain damage in wild deer populations in Colorado and Wyoming.

1:10:23.800 --> 1:10:26.719
<v Speaker 1>Since then, it has spread every year throughout the American

1:10:26.760 --> 1:10:32.440
<v Speaker 1>West Plains and more recently the Midwest and Canada, Saskatchewan

1:10:32.520 --> 1:10:37.160
<v Speaker 1>and Alberta. Chronic wasting may pose a threat to other

1:10:37.280 --> 1:10:42.000
<v Speaker 1>ungulate species as well. It's been spoted in an elk, moose, reindeer, etc.

1:10:42.600 --> 1:10:45.240
<v Speaker 1>And to other parts of the world. It's been seen

1:10:45.320 --> 1:10:48.880
<v Speaker 1>in South Korea, Norway and Finland, for instance. How is

1:10:48.920 --> 1:10:53.080
<v Speaker 1>it spreading? Deer aren't forced to eat deer meat, and

1:10:53.120 --> 1:10:57.040
<v Speaker 1>they aren't in as high of population densities as sheep.

1:10:57.280 --> 1:11:01.599
<v Speaker 1>Prions are incredibly tough, so it's proba that chronic wasting

1:11:01.720 --> 1:11:05.400
<v Speaker 1>is transmitted among these ungulate species by direct contact via

1:11:05.439 --> 1:11:11.160
<v Speaker 1>bodily fluids, indirect contact through a contaminated environment, or by

1:11:11.200 --> 1:11:14.439
<v Speaker 1>a crow acting as a vector for dispersal of the preon.

1:11:14.600 --> 1:11:16.160
<v Speaker 2>Weird a crow?

1:11:16.360 --> 1:11:19.719
<v Speaker 1>Yeah, so a crow, I don't know. Basically, a crow

1:11:19.760 --> 1:11:21.759
<v Speaker 1>eats a bit of this is this is in terms

1:11:21.880 --> 1:11:23.320
<v Speaker 1>of long distance dispersal.

1:11:23.840 --> 1:11:24.320
<v Speaker 2>I don't know.

1:11:24.400 --> 1:11:26.200
<v Speaker 1>The crow eats a bit of a deer that died

1:11:26.240 --> 1:11:29.920
<v Speaker 1>of chronic wasting. The preon survives the digestive journey and

1:11:29.960 --> 1:11:32.280
<v Speaker 1>then it poops it out to contamine it a different

1:11:32.320 --> 1:11:33.080
<v Speaker 1>part of the forest.

1:11:33.360 --> 1:11:35.759
<v Speaker 2>Seems a little sas, but I mean.

1:11:35.720 --> 1:11:40.240
<v Speaker 1>I'm not sure i'd yeah, but this is still an

1:11:40.280 --> 1:11:42.639
<v Speaker 1>area of very active and interesting research.

1:11:42.920 --> 1:11:46.280
<v Speaker 2>People at Illinois doing cool research on chronic wasting disease.

1:11:46.960 --> 1:11:51.400
<v Speaker 1>Yeah. The whole time I was researching preons, I kept

1:11:51.400 --> 1:11:53.960
<v Speaker 1>getting this feeling that I was just seeing the tip

1:11:54.000 --> 1:11:57.599
<v Speaker 1>of the iceberg. We obviously know so much more about

1:11:57.600 --> 1:12:00.800
<v Speaker 1>preons today than fifty years ago, but so much seems

1:12:00.880 --> 1:12:04.120
<v Speaker 1>left to be uncovered. It truly is a new frontier

1:12:04.240 --> 1:12:06.800
<v Speaker 1>of health and medical research, and it's changed the way

1:12:06.800 --> 1:12:11.000
<v Speaker 1>we think about infection. Like we talked about prions don't

1:12:11.000 --> 1:12:14.760
<v Speaker 1>have this biological imperative to pass down their genes. It's

1:12:14.880 --> 1:12:18.799
<v Speaker 1>just a protein, and yet it is an infectious disease

1:12:19.760 --> 1:12:22.200
<v Speaker 1>and one that has told us a lot about ourselves.

1:12:22.720 --> 1:12:25.680
<v Speaker 1>So what I want to know is A, what do

1:12:25.760 --> 1:12:30.000
<v Speaker 1>the numbers look like today? And B what's the latest

1:12:30.160 --> 1:12:34.559
<v Speaker 1>on cures or treatments or technologies or whatever.

1:12:35.920 --> 1:13:10.040
<v Speaker 2>Yeah, Okay, it's interesting that you described this as like

1:13:10.200 --> 1:13:15.479
<v Speaker 2>a new frontier of research kind of because even in

1:13:15.600 --> 1:13:18.280
<v Speaker 2>just hearing what you were just talking about with chronic

1:13:18.320 --> 1:13:22.920
<v Speaker 2>wasting and what I've read about what's going on in

1:13:23.000 --> 1:13:28.320
<v Speaker 2>research today, it seems like there's a lot of open

1:13:28.439 --> 1:13:32.479
<v Speaker 2>territory and possibilities for research. If I wanted to keep

1:13:32.520 --> 1:13:38.040
<v Speaker 2>doing research, maybe I'd switched to preons right now. But

1:13:38.200 --> 1:13:41.040
<v Speaker 2>in terms of the research that's going on, it seems like,

1:13:41.320 --> 1:13:44.760
<v Speaker 2>and I'm probably missing a lot of it, but it

1:13:44.800 --> 1:13:49.599
<v Speaker 2>seems like there's kind of two main branches, because there's

1:13:49.640 --> 1:13:52.720
<v Speaker 2>two big areas that we still lack a lot of

1:13:52.720 --> 1:13:56.120
<v Speaker 2>information on. One of those areas is focused on actually

1:13:56.200 --> 1:14:02.040
<v Speaker 2>understanding the mechanism behind this path of physiology of disease,

1:14:02.720 --> 1:14:04.719
<v Speaker 2>and a lot of that has to do with figuring

1:14:04.720 --> 1:14:07.400
<v Speaker 2>out what this protein does in our body when it's

1:14:07.439 --> 1:14:11.800
<v Speaker 2>not misfolded. So because we still don't entirely know what

1:14:11.880 --> 1:14:15.880
<v Speaker 2>this normal protein does. It's a protein that is very

1:14:15.920 --> 1:14:20.640
<v Speaker 2>abundant in neuron cell plasma membranes, and there's a lot

1:14:20.680 --> 1:14:24.200
<v Speaker 2>of thought that it has to do with possibly calcium channels,

1:14:24.439 --> 1:14:29.280
<v Speaker 2>possibly copper, maybe bringing things into cells or pushing ions

1:14:29.320 --> 1:14:33.040
<v Speaker 2>out of cells. It's not entirely clear. It does seem

1:14:33.080 --> 1:14:35.240
<v Speaker 2>to be a really important protein, and it makes sense

1:14:35.240 --> 1:14:37.960
<v Speaker 2>that it's in such high density on your neurons since

1:14:38.000 --> 1:14:41.479
<v Speaker 2>those seem to be so highly affected. But we still

1:14:41.479 --> 1:14:44.120
<v Speaker 2>don't know, Like there is no clear answer as to

1:14:44.240 --> 1:14:49.840
<v Speaker 2>what this protein does. The other field of research is

1:14:51.120 --> 1:14:53.120
<v Speaker 2>what do we do about it? We know that this

1:14:53.240 --> 1:14:55.639
<v Speaker 2>disease exists, so how do we treat it?

1:14:57.160 --> 1:15:04.160
<v Speaker 3>And yeah, yeah, So at this point it doesn't seem

1:15:04.560 --> 1:15:07.920
<v Speaker 3>like much of that research in that realm is out

1:15:07.960 --> 1:15:12.080
<v Speaker 3>of the screening phase, or maybe at best the animal

1:15:12.160 --> 1:15:18.519
<v Speaker 3>model's phase. So a tiny background on drug development. Any

1:15:18.600 --> 1:15:21.080
<v Speaker 3>drug that gets developed, especially if it's a brand new

1:15:21.160 --> 1:15:23.040
<v Speaker 3>drug and not a drug that we use for a

1:15:23.080 --> 1:15:25.320
<v Speaker 3>different disease that you want to use to treat some

1:15:25.439 --> 1:15:28.879
<v Speaker 3>other disease, it goes through a lot of stages of screening.

1:15:29.439 --> 1:15:32.080
<v Speaker 3>Usually it's tested in cell culture first to make sure

1:15:32.120 --> 1:15:33.960
<v Speaker 3>it does the thing that you're trying to get it

1:15:34.000 --> 1:15:37.639
<v Speaker 3>to do, and then you screen it. You often screen

1:15:37.640 --> 1:15:39.759
<v Speaker 3>a whole bunch of different compounds and you say, okay,

1:15:40.040 --> 1:15:42.160
<v Speaker 3>which one of these compounds has an effect on this

1:15:42.240 --> 1:15:47.879
<v Speaker 3>protein or against this pathogen. So there's a lot of papers,

1:15:47.960 --> 1:15:50.839
<v Speaker 3>especially from the early two thousands, where they were screening

1:15:50.960 --> 1:15:54.000
<v Speaker 3>hundreds or sometimes thousands of compounds to see if any

1:15:54.040 --> 1:15:57.519
<v Speaker 3>of them had an effect on diminishing the accumulation of

1:15:57.600 --> 1:16:02.680
<v Speaker 3>misfolded preon proteins. Okay, So there was at least some

1:16:02.720 --> 1:16:05.000
<v Speaker 3>work that has been done so far and is i'm

1:16:05.040 --> 1:16:08.040
<v Speaker 3>sure continuing to be done to try and just find compounds.

1:16:08.560 --> 1:16:11.840
<v Speaker 3>Once they find those promising compounds, then you test those

1:16:11.920 --> 1:16:15.320
<v Speaker 3>on animals, usually in mice, but also in other animals

1:16:15.800 --> 1:16:19.240
<v Speaker 3>as well to see if they're safe and if they

1:16:19.320 --> 1:16:21.320
<v Speaker 3>actually work once you take them out of a cell

1:16:21.360 --> 1:16:25.639
<v Speaker 3>culture and put them into a real animal. Then once

1:16:25.680 --> 1:16:28.160
<v Speaker 3>you can show that a compound actually works on a

1:16:28.200 --> 1:16:30.599
<v Speaker 3>disease in an animal, you have to test it on

1:16:30.680 --> 1:16:32.880
<v Speaker 3>small groups of humans to make sure that it doesn't

1:16:32.960 --> 1:16:37.200
<v Speaker 3>kill people or cause any other serious reactions. And that's

1:16:37.240 --> 1:16:39.880
<v Speaker 3>a phase one trial, and then you do a phase

1:16:39.920 --> 1:16:42.240
<v Speaker 3>two trial, which is a larger group of humans, and

1:16:42.280 --> 1:16:44.360
<v Speaker 3>now you want to look at does it actually work.

1:16:44.520 --> 1:16:46.640
<v Speaker 3>Like we know it doesn't kill you, now we can

1:16:46.680 --> 1:16:48.960
<v Speaker 3>ask does it actually work? Then you have to do

1:16:49.040 --> 1:16:52.080
<v Speaker 3>even bigger trials. So it's a very very long process

1:16:52.360 --> 1:16:55.400
<v Speaker 3>before you actually get any sort of drug to market,

1:16:56.280 --> 1:16:59.960
<v Speaker 3>and as far as I can tell, any potential drugs

1:17:00.080 --> 1:17:03.760
<v Speaker 3>seem to be in the very early stages. Still.

1:17:03.840 --> 1:17:09.240
<v Speaker 2>There are some drugs or some treatments that have been

1:17:09.400 --> 1:17:14.120
<v Speaker 2>used to treat other diseases. There's an anahistamine which is

1:17:14.200 --> 1:17:17.280
<v Speaker 2>super random. Anahistamine is like benadryl. It's like what you

1:17:17.320 --> 1:17:21.360
<v Speaker 2>take if you have allergies or something like that. Yeah,

1:17:21.600 --> 1:17:26.080
<v Speaker 2>that in those early trials of trying to identify compounds,

1:17:26.600 --> 1:17:33.920
<v Speaker 2>seemed to have effect on binding to Alzheimer's proteins, misfolded

1:17:33.920 --> 1:17:37.320
<v Speaker 2>Alzheimer's proteins, so it was suggested that perhaps it also

1:17:37.560 --> 1:17:43.599
<v Speaker 2>could bind too misfolded preon proteins. Oh, it has been

1:17:43.600 --> 1:17:47.400
<v Speaker 2>shown in cell culture also to inhibit the preon protein,

1:17:47.880 --> 1:17:51.240
<v Speaker 2>but I haven't I haven't found any papers that it

1:17:51.720 --> 1:17:54.160
<v Speaker 2>has been tested on beyond the cell culture level. But

1:17:54.200 --> 1:17:58.080
<v Speaker 2>that's a drug that already exists and has been used

1:17:58.080 --> 1:18:00.400
<v Speaker 2>in humans. It's not generally used because there's a lot

1:18:00.400 --> 1:18:05.719
<v Speaker 2>of side effects. But you know, whenever we look at drugs,

1:18:05.760 --> 1:18:08.360
<v Speaker 2>we have to look at the side effects versus the disease.

1:18:08.479 --> 1:18:12.040
<v Speaker 2>And if you're treating something as benign as allergies, you

1:18:12.200 --> 1:18:13.960
<v Speaker 2>want to make sure you have very little side effects.

1:18:14.000 --> 1:18:16.360
<v Speaker 2>But if you're treating something where the only alternative is death,

1:18:16.400 --> 1:18:19.479
<v Speaker 2>you might tolerate a lot more side effects. So who knows.

1:18:20.760 --> 1:18:25.439
<v Speaker 2>There's another treatment called PPS, which is pentocin polysulfate that

1:18:25.560 --> 1:18:30.280
<v Speaker 2>it's been getting some press and trying to treat preon diseases,

1:18:31.240 --> 1:18:33.880
<v Speaker 2>but it has not been shown to work. So people

1:18:33.880 --> 1:18:36.200
<v Speaker 2>have actually tried this in at least a couple of

1:18:36.240 --> 1:18:39.000
<v Speaker 2>humans and they died. It didn't work at all.

1:18:39.880 --> 1:18:41.280
<v Speaker 1>So but it's still getting pressed.

1:18:41.400 --> 1:18:44.320
<v Speaker 2>It's still getting press, or at least it got press.

1:18:44.360 --> 1:18:47.120
<v Speaker 2>I don't know if it's still getting press. There's at

1:18:47.200 --> 1:18:50.200
<v Speaker 2>least one study that suggests that it delays the progression

1:18:50.200 --> 1:18:53.960
<v Speaker 2>of disease and decreases the amount of abnormal protein deposition

1:18:54.160 --> 1:18:58.480
<v Speaker 2>in mice brains, but you had to inject it directly

1:18:58.640 --> 1:19:04.880
<v Speaker 2>into the mouse, so that's not likely something that's going

1:19:04.960 --> 1:19:09.360
<v Speaker 2>to be in human trial soon. But yeah, yeah, So

1:19:09.960 --> 1:19:13.720
<v Speaker 2>I don't know. It seems like there's a lot of

1:19:14.200 --> 1:19:16.599
<v Speaker 2>open territory, and I do think that a lot of

1:19:16.600 --> 1:19:18.880
<v Speaker 2>this kind of hinges on being able to figure out

1:19:19.240 --> 1:19:23.200
<v Speaker 2>this protein. Because with a lot of other diseases, even

1:19:23.240 --> 1:19:27.800
<v Speaker 2>if we don't fully understand everything about a bacterium or

1:19:27.880 --> 1:19:32.000
<v Speaker 2>a virus, we know generally how they replicate, We know

1:19:32.280 --> 1:19:36.639
<v Speaker 2>generally you know how they operate, and so we can

1:19:36.840 --> 1:19:41.040
<v Speaker 2>use drugs that we have to try and stop them.

1:19:41.520 --> 1:19:44.360
<v Speaker 2>There are targets that we know that we can aim

1:19:44.439 --> 1:19:47.760
<v Speaker 2>for But with this, it's it's really hard and it is,

1:19:47.800 --> 1:19:50.000
<v Speaker 2>like you said early on, it's a protein that's in

1:19:50.080 --> 1:19:54.599
<v Speaker 2>our bodies, and presumably it's an important one, you know, right,

1:19:54.720 --> 1:19:58.880
<v Speaker 2>it has to be. Yeah, In terms of numbers, the

1:19:58.920 --> 1:20:01.400
<v Speaker 2>only one where there's you can get sort of good

1:20:01.520 --> 1:20:05.840
<v Speaker 2>numbers on is classic cruise felt yakup disease. And so

1:20:06.400 --> 1:20:09.720
<v Speaker 2>the majority of those cases eighty five percent are sporadic,

1:20:10.120 --> 1:20:14.760
<v Speaker 2>about five to fifteen percent are inherited, and overall those

1:20:15.360 --> 1:20:19.519
<v Speaker 2>are at a rate of about one to three cases

1:20:20.240 --> 1:20:22.639
<v Speaker 2>per million people per year.

1:20:23.240 --> 1:20:25.679
<v Speaker 1>Oh, it's quite rare. Okay, Yeah, it's pretty rare.

1:20:26.400 --> 1:20:30.040
<v Speaker 2>So in the US, for example, there have been eleven

1:20:30.080 --> 1:20:34.479
<v Speaker 2>thousand cases reported from nineteen seventy nine to twenty sixteen,

1:20:35.120 --> 1:20:37.840
<v Speaker 2>so between yeah, between one hundred and eighty and four

1:20:37.920 --> 1:20:41.840
<v Speaker 2>hundred cases per year. And when you look so on

1:20:41.880 --> 1:20:46.000
<v Speaker 2>the CDC's website, you can find a chart essentially of

1:20:46.040 --> 1:20:48.120
<v Speaker 2>the number of cases that have been reported in the

1:20:48.240 --> 1:20:52.960
<v Speaker 2>US each year of classic CJD, and the numbers get

1:20:52.960 --> 1:20:55.200
<v Speaker 2>bigger every year. But it's important to keep in mind

1:20:55.240 --> 1:20:58.000
<v Speaker 2>that it could be in part due to better screening

1:20:58.040 --> 1:21:01.639
<v Speaker 2>and recognition. It also could be because our population size

1:21:01.640 --> 1:21:04.160
<v Speaker 2>has grown a lot since nineteen seventy nine. So when

1:21:04.200 --> 1:21:07.320
<v Speaker 2>you see four hundred verses one twenty, it's sometimes easy

1:21:07.320 --> 1:21:10.360
<v Speaker 2>to go, oh my god, it's growing, and it's but

1:21:10.560 --> 1:21:12.679
<v Speaker 2>it actually has been at a pretty constant rate.

1:21:13.479 --> 1:21:17.080
<v Speaker 1>I've also read that a lot of or at least

1:21:17.080 --> 1:21:20.560
<v Speaker 1>like a proportion of CJD patients have been misdiagnosed as Alzheimer's.

1:21:20.600 --> 1:21:24.799
<v Speaker 2>Absolutely yeah, because with CJD you present with similar symptoms.

1:21:24.800 --> 1:21:28.280
<v Speaker 2>You present with, you know, rapid onset dementia. I think

1:21:28.360 --> 1:21:31.120
<v Speaker 2>the difference is the onset of time and then the

1:21:31.160 --> 1:21:34.879
<v Speaker 2>time to death. Alzheimer's is a very very slow progressing disease.

1:21:34.960 --> 1:21:39.840
<v Speaker 2>Generally it's faster if it's earlier onset. This is what

1:21:39.920 --> 1:21:40.680
<v Speaker 2>I want to end on.

1:21:42.040 --> 1:21:42.400
<v Speaker 1>Okay.

1:21:45.280 --> 1:21:52.439
<v Speaker 2>I think that chronic wasting disease is so important, and

1:21:52.479 --> 1:21:54.720
<v Speaker 2>I hope that I know that there are so many

1:21:54.720 --> 1:21:57.519
<v Speaker 2>people studying it, but I I just want them to

1:21:57.520 --> 1:22:01.720
<v Speaker 2>get so much money for research because be cuse so

1:22:01.800 --> 1:22:05.000
<v Speaker 2>there have been no there has been so far no

1:22:05.160 --> 1:22:10.080
<v Speaker 2>evidence that chronic wasting disease in deer can cause disease

1:22:10.160 --> 1:22:12.879
<v Speaker 2>in humans. There have been at least a few studies

1:22:12.920 --> 1:22:16.000
<v Speaker 2>that have looked for those links. Because here's the thing

1:22:16.400 --> 1:22:20.920
<v Speaker 2>about chronic wasting. Even though we haven't yet found any

1:22:20.960 --> 1:22:25.200
<v Speaker 2>evidence that chronic wasting disease in deer can cause disease

1:22:25.280 --> 1:22:30.560
<v Speaker 2>in humans, it has been found at very high prevalence

1:22:30.680 --> 1:22:34.920
<v Speaker 2>in deer populations. In wild deer populations rates up to

1:22:35.040 --> 1:22:39.320
<v Speaker 2>ten or twenty five percent, but in captive herds up

1:22:39.400 --> 1:22:44.400
<v Speaker 2>to eighty percent of deer have been found to be infected.

1:22:45.600 --> 1:22:49.280
<v Speaker 2>And if you combine that with data from the CDC,

1:22:49.640 --> 1:22:52.920
<v Speaker 2>that suggests that up to twenty percent of people surveyed

1:22:53.080 --> 1:22:56.720
<v Speaker 2>had hunted deer at some point, and up to two

1:22:56.920 --> 1:22:59.920
<v Speaker 2>thirds of Americans have eaten venice.

1:23:00.080 --> 1:23:04.360
<v Speaker 1>In if it I've eaten venison, I've eaten venison.

1:23:04.479 --> 1:23:08.799
<v Speaker 2>Our neighbor hunts deer, breaks them over, and there's chronic

1:23:08.840 --> 1:23:17.200
<v Speaker 2>wasting disease in Illinois. I find the prospect terrifying.

1:23:18.160 --> 1:23:22.120
<v Speaker 1>Yeah, tip of the iceberg, Tip of the iceberg.

1:23:23.600 --> 1:23:28.360
<v Speaker 2>It's so interesting because it's not outside the rum of possibility. Right,

1:23:28.439 --> 1:23:33.000
<v Speaker 2>we know that it can happen from eating contaminated cow meat,

1:23:33.680 --> 1:23:36.799
<v Speaker 2>right because I don't think there have been any cases

1:23:36.840 --> 1:23:39.639
<v Speaker 2>of humans getting any kind of preon disease from eating

1:23:40.200 --> 1:23:43.120
<v Speaker 2>sheep meat infected with scrapy. Is that correct?

1:23:43.240 --> 1:23:44.240
<v Speaker 1>That's what it seems to be.

1:23:44.360 --> 1:23:48.240
<v Speaker 2>Yes, And so I'm curious if maybe it's because the

1:23:48.680 --> 1:23:52.759
<v Speaker 2>outbreaks of variant CJD that were from mad cow disease.

1:23:53.160 --> 1:23:55.479
<v Speaker 2>I wonder if it's just that the prions were in

1:23:55.600 --> 1:23:58.240
<v Speaker 2>higher concentration in the cow because of the ways that

1:23:58.280 --> 1:24:01.120
<v Speaker 2>the cows got infected, so then now they're in higher

1:24:01.160 --> 1:24:02.280
<v Speaker 2>concentrations in humans.

1:24:02.400 --> 1:24:02.800
<v Speaker 1>I don't know.

1:24:02.920 --> 1:24:04.400
<v Speaker 2>I don't think anybody knows.

1:24:04.520 --> 1:24:08.679
<v Speaker 1>Right something about the folding of that particular fold. Who knows?

1:24:08.960 --> 1:24:12.800
<v Speaker 2>Yeah, right, yeah, who knows? I want to know, though.

1:24:13.040 --> 1:24:16.439
<v Speaker 1>I know, I know, yeah, you know. And kind of

1:24:16.520 --> 1:24:19.759
<v Speaker 1>in retrospect, despite that being one of the longest histories

1:24:19.840 --> 1:24:25.519
<v Speaker 1>that we've ever discussed, it feels premature because it seems

1:24:25.560 --> 1:24:28.760
<v Speaker 1>like there's so much more to be done and that

1:24:28.800 --> 1:24:30.599
<v Speaker 1>there's so much more that's going to be discovered.

1:24:30.840 --> 1:24:34.360
<v Speaker 2>Yeah, And I feel like in talking about what's currently happening,

1:24:34.439 --> 1:24:37.600
<v Speaker 2>it's kind of just like so many things, but I

1:24:37.600 --> 1:24:39.880
<v Speaker 2>don't know how to talk about any of them because

1:24:39.880 --> 1:24:42.280
<v Speaker 2>they're all kind of in their infancy, like there's no

1:24:43.640 --> 1:24:46.799
<v Speaker 2>you know, there's no here's the new drug or here's

1:24:46.960 --> 1:24:50.639
<v Speaker 2>we have an answer. It's all just currently being figured out,

1:24:50.680 --> 1:24:52.640
<v Speaker 2>which I find so exciting.

1:24:53.479 --> 1:24:59.320
<v Speaker 1>Yeah, it's super super like cutting edge. Yeah, news is

1:24:59.360 --> 1:25:00.320
<v Speaker 1>happening right.

1:25:01.600 --> 1:25:04.200
<v Speaker 2>Yeah. On that note, should we share our sources?

1:25:04.760 --> 1:25:09.559
<v Speaker 1>Yeah, great idea. So I read a couple of books.

1:25:09.640 --> 1:25:12.360
<v Speaker 1>One is called Madness and Memory and that is by

1:25:12.439 --> 1:25:16.240
<v Speaker 1>Stanley Prusner, so that is his memoir of pre on research.

1:25:17.000 --> 1:25:19.640
<v Speaker 1>I also read The Family That Couldn't Sleep by Dtmax,

1:25:20.040 --> 1:25:24.680
<v Speaker 1>great overview of preon history. And then I have a

1:25:24.680 --> 1:25:28.519
<v Speaker 1>few articles which I will put on our sources list.

1:25:28.600 --> 1:25:31.280
<v Speaker 1>And then finally, if you're interested, there is a documentary

1:25:31.320 --> 1:25:35.720
<v Speaker 1>about Carlton Gadgetsek called The Genius and the Boys.

1:25:35.960 --> 1:25:39.760
<v Speaker 2>I have so many articles they're all going to be

1:25:39.760 --> 1:25:42.400
<v Speaker 2>posted on our website, this podcast will Kill You dot

1:25:42.439 --> 1:25:45.280
<v Speaker 2>Com under the episodes tab, so we post all of

1:25:45.320 --> 1:25:49.240
<v Speaker 2>our sources from every single episode, this one in particular.

1:25:49.560 --> 1:25:53.840
<v Speaker 2>I have a lot of cool articles about the current

1:25:53.880 --> 1:25:54.920
<v Speaker 2>research that's being done.

1:25:54.960 --> 1:25:56.479
<v Speaker 1>So if you're interested.

1:25:56.120 --> 1:26:00.000
<v Speaker 2>In reading more about what's going on with pre on research, now,

1:26:00.120 --> 1:26:07.439
<v Speaker 2>definitely check that out. Cool. Yeah, Well, thank you everyone

1:26:07.479 --> 1:26:11.720
<v Speaker 2>for listening. We couldn't make a podcast without you. Well

1:26:11.720 --> 1:26:16.160
<v Speaker 2>we could, but then it would be just us, be weird.

1:26:16.840 --> 1:26:20.840
<v Speaker 1>It would be weird. Thanks to Bloodmobile for providing the

1:26:20.920 --> 1:26:22.920
<v Speaker 1>music for this and all of our episodes.

1:26:23.200 --> 1:26:24.679
<v Speaker 2>Thanks thanks so much.

1:26:26.920 --> 1:26:29.400
<v Speaker 1>Well, on that note, wash your hands

1:26:29.960 --> 1:26:31.720
<v Speaker 2>You filthy animals.