The Disease You Will Never Survive
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- Опубліковано 2 чер 2024
- Check out DeepMind's AlphaFold - and marvel at the complex 3D structure of proteins - at alphafold.ebi.ac.uk/
A simple mis-folding in a certain brain protein causes a disease for which we have no cure.
LEARN MORE
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To learn more about this topic, start your googling with these keywords:
- Prion: an abnormal pathogenic agent that is transmissible and able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain.
- Spongiform encephalopathies: a group of rare degenerative brain disorders caused by prions and characterized by tiny holes that give the brain a “spongy” appearance.
- Amyloid: an abnormal aggregate of protein.
- Creutzfeldt-Jakob disease: also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is an invariably fatal degenerative brain disorder. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. About 70% of people die within a year of diagnosis.
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CREDITS
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Lizah van der Aart | Script Writing, Narration, Illustration, Video Editing and Animation
Kate Yoshida | Script Editor and Video Director
Nathaniel Schroeder | Music
A huge thanks to Dr. Emmanuelle Vire for sharing her expertise on prion biology and epigenetics.
MinuteEarth is produced by Neptune Studios LLC
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Lizah van der Aart • Sarah Berman • Cameron Duke
Arcadi Garcia i Rius • David Goldenberg • Melissa Hayes
Alex Reich • Henry Reich • Peter Reich
Ever Salazar • Leonardo Souza • Kate Yoshida
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REFERENCES
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Dabin LC, Guntoro F, Campbell T, Bélicard T, Smith AR, Smith RG, Raybould R, Schott JM, Lunnon K, Sarkies P, Collinge J, Mead S, Viré E (2020). Altered DNA methylation profiles in blood from patients with sporadic Creutzfeldt-Jakob disease. Acta Neuropathology 140(6):863-879. doi.org/10.1007/s00401-020-02...
Miller G. (2009) Neurodegeneration. Acting like a prion isn't always bad. Science 326 (5958):1338. doi.org/10.1126/science.326.5...
Quartararo, AJ. (2020) Synthesis of Proteins by Automated Flow Chemistry. Science 368 (6494): 980- 987, doi.org/10.1126/science.abb2491
Sabate R. (2014) When amyloids become prions. Prion 8 (3):233-9. doi.org/10.4161/19336896.2014...
Scheckel, C., Aguzzi, A. (2018) Prions, prionoids and protein misfolding disorders. Nature Reviews Genetics 19, 405-418. doi.org/10.1038/s41576-018-00...
Vastag, B. (2009) The beneficial side of prions. Nature doi.org/10.1038/news.2009.23 - Наука та технологія
Lizah has been an important part of the team for a little more than a year. However, this is the first time she has written and narrated a MinuteEarth video (on top of illustrating it!). We all hope you like it as much as we do! - Ever
I really liked the anime/video-game references lol. Knowledgeable and great taste too!
I'm a non-native English-speaker and the voice was still really easy to understand. So, that's a welcome addition to your narrators ^^
As for the content, its quality is on par with the other videos. 👍
Pronunciation was child-like kind of cute lol.. so.. keep doing these hehe
Im confused what does this have to do with prisons? Cool video though
Edit: ohhhhh, it says prions. 😂
Shes really good at explaining. I hope we see more of her!
I remember a speech given by a neurosurgeon about prion disease. He needed to do nurosurgery on someone with CJD. He said it was the first time he ever triple-gloved for a surgery.
Rumor has it they destroy all surgical instruments used on someone with CJD. Nobody in charge wants to take chances in sterilization not being perfect.
Seems about right, since other deer have gotten CWD from breathing the same air or even touching an infected deer. CWD is Chronic Wasting Disease, a prion that affects deer.
@@galehunter2519 CJD is 'Creutzfeldt-Jakob disease'. CWD is something different.
@@RO_Tim Didn't the video say they are both different types of prions? Presumably they may have similar behavior
@@RO_Tim The video literally says it's a variant of the same condition with slight shape variations
My mom had a patient with CJD. It was hard to watch because the patient was *well* aware and terrified of the fact that she was rapidly forgetting things, like what a spoon is, or her parents' faces. With dementia caused by, say, Alzheimer's it happens slowly enough that usually the person doesn't realize the extent of change, but not with CJD.
Yeah. Being aware you're losing your cognitive function sounds utterly terrifying to me. I'd say it's a worse fate than dying.
Idk which is worse, prion disease or rabies- both haunt my nightmares, in the form or flashbacks from the one experience of my own brain truly screwing up big time, in the form of alcohol withdrawal- with delirium tremens you know you're fading fast but can't get help in time.
But at least I was just one shot of Ativan away from being okay I can't imagine being STUCK in that kind of state for days or months, knowing "this his how I'm gonna die" NOOOOO
@@callummclachlan4771 i mean… you are actually dying when you have a prion dieases
@@90klh Rabies isn't nearly as bad because it only kills if you have no idea you were infected. Once you get a prion infection, you're f**ked no matter what
@@Alec____ people can get rabies without knowing they got bitten.
I'll grant you that it's spooky that prions can spontaneously pop up, but I'm talking about the symptoms themselves, cuz once you get that first symptom, a fever, a headache, or a little malaise from rabies, your already dead. Vaccines won't save you at that point and neither will any other treatment
The weirdest thing to me about this is that the way a protein is folded... is *contagious*. Like, it’s not an organism, it’s just a thing, but it can still infect other proteins it encounters somehow...
I mean, they're not doing it intentionally. It's easy to call them evil or imagine that they're acting maliciously, but its literally just a misfolded protein interacting differently when it contacts another one
@@whyonthefall5373 it's the lack of intent that makes it weird. It's like if your neatly coiled headphones (before we all had to get wireless ones, of course) got tangled up just because you put them next to a tangled pair.
And on the atomic level there's strange matter (theoretically)
@Richard Winskill it's more like your neatly shaped square of magnets gets close to a circle of magnets, which because of physics both get shaped like a circles of magnets.
Precursor to life, anyone?
My wife’s mother died from CJD. It was incredibly hard to witness the cognitive decline over a matter of months.
My condolences, thats a scary experience. ❤
In English, your wife's mother is called your mother in law.
o7
How did your family find out? I mean were there odd symptoms that went misdiagnosed at first ? (If you don't mind me asking)
@@90klh Don’t mind at all. Early signs were very similar to Parkinson’s disease. Degraded motor function coupled with rapidly increasing memory loss were the notable indicators. In fact, Parkinson’s was the initial diagnosis from her doctor.
The reason we pushed for a second opinion regarding CJD was because my wife’s grandmother died of the same cause (though it was unfortunately never tested postmortem, which would have allowed us to identify the gene sooner). When her mother passed, she was tested to confirm it to be the genetic variant of CJD, which unfortunately means that my wife and her sisters also have an increased risk of carrying it as well.
Now that we have the genetic profile available, my wife will be able to test for it. It’s something we do plan to find out soon, but it’s been hard. Obviously her whole outlook on life will change as a result of that test, so it hasn’t been easy. I give her a lot of credit for the work she put in under incredibly hard circumstances. Her and I were only 19 when she lost her mom, yet she had the strength to coordinate this as well as her funeral. I give her a lot of props for that.
Hope this helps.
My mom is currently suffering from this. Its awful. Its one of those things you would not wish on your worst enemy. I tell my mom I love her every night and I pray she is still able to recognize my voice in her current state.
I’m so sorry that she’s going through this, I hope she’s in the least pain possible. 😔🙏🏽❤
Thats One of the hardest diseases for a caregiver to face off. Even harder if its hurting a parent. You have all my simpathy. Dont be afraid to seek help or istitutionalize her if U feel u cant do this anymore. It can easily burn out U psichologically. Much respect from an Italian Nurse
@@rawdata678 She passed away tuesday morning in the arms of my dad. She went from completely normal, losing all her faculties and passing away in the span of 3 months.I'm glad she's no longer suffering and is now with her brother and father. My dad is crushed right now, him and mom we're together for 35 years. I'm sad she's gone but comforted knowing we'll all be reunited one day. Thank you for your kind words.
@@loosesingularity3121 probably doesnt mean much from a stranger but my condolences for your loss
@@loosesingularity3121 much respect and love
Correction:
CJD is not fast killer at all. Only the symptomatic phase is short and deadly. Asymptomatic phase can take many many years.
thanks 💀
Help
@@h0eera.115 ?
well this is concerning
@@callyral Why?
And, BTW, a note about MinuteX channel family: here a video on a topic in molecular biology is written, illustrated and narrated by an _actual PhD_ in molecular biology, who just happens to also be a talented communicator, graphics artist and whatever other hidden talents Lizah has. This is actually far rarer "phenomenon" on YT and elsewhere than it should be.
Simp
I genuely thought it was Lisa Since she sounds like she is from The Netherlands however it is indeed Lizah. Lisa is way more common in The Netherlands.
@@Pleezath hah yeah I have some weird spelling going on :’)
Aaw thank you so much ❤ it was quite a process and I am so happy with how it turned out
actually i think they're more common than you might think. A lot of my friends who planned to be a doctor are good note-taker, their notes are just very precise and well illustated. It's better for them to learning in that way, since biology-related things are better to comprehend using illustation and graphics.
The idea that getting CJD is as likely as getting struck by lightning is terrifying.
Fatal familial insomnia is probably the worse prion in a few weeks going from a normal person to being completely unable to sleep and dying because your body cant take the stress ironically is the stuff of nightmares.
I remember reading about that and then being terrified for weeks about it, not knowing if it would ever happen to me or a loved one.
In America, your odds of dying from lightning in a given year, is less than one in a million.
Your odds of dying in a car crash, about 1 in 103 per year.
Unless you absolutely, AND literally, pee yourself at the thought of getting into a car, you really have no reason to even think about things like this.....
@@lordgarion514 There's no way the probability is that high. That would make car accidents the number one cause of death.
@@lordgarion514 That’s the probability of dying once in a car crash not the probability of dying in a car crash as a whole.
@@matth227 im so sorry you phrased your comment weirdly, dying "once" in a car crash? Dont we all die once?
This was one of my random childhood fears. I was super scared (I got panic attacks and anxiety) that I would develop prions.
think hard enough and the brain might make it there mate!
I fear it now as an adult.
Me after playing Plague Inc:
Me asf after eating hot pockets including the plastic wrapper it comespakcygatedwithh
@@BoxOfCurryos did you have a stroke in the middle of typing that?
Me: does everything not to die
My brain: *"fine i'll do it myself"*
🤣🤣
or better, "does everything to not die"
Who is I?
The brain is you though
And sometimes the brain commands unaliving itself instantly. Guys, I thing that we should find a better commander
My great grandpa died of Creutzfeldt-Jakob. It was before I was born, so I didn't see the decline, but according to my mom it was very fast. It's one of the diseases that simultaneously fascinates and terrifies me.
The terrifying thing is that whilst CJD is rare, the tainted beef scandal of the 80’s and 90’s and the long period before CJD causes symptoms in humans means that we may not see a number of cases for the next sixty years or so and end up with a sudden explosion of cases.
It takes like, under a decade to show symptoms last I checked. Under two, at most.
Unless it's the familial version*
20 years max is the projected time of death. So seems like we past the peak a few years ago.
If you check the data on cases you can see it already peaked. It was completely terrifying as a kid at the time, we were told we weren't allowed to eat beef anymore, it was all over the news.
@@Jess-T For the time being. We don’t know whether there will be an explosion of cases in the future yet or further pockets of infection.
I don’t have the energy to be terrified of a disease I could do nothing to predict and could do nothing to treat if infected. I’ve got enough anxieties. At this point I’m just kind of fascinated.
I hate how this is how I feel, too. I’m just so desensitized at this point, lmao
That's a beautiful way to look at fears in general
My mom was in Europe during the mad cow disease scare. And even though it's been decades and she hasn't exhibited any signs of it, it still makes me extremely worried and scared. I love my mom more than anything, and the thought of losing her so fast makes me cry. I hope we can come up with a cure for this one day and even make a preventative treatment.
I live in Europe in the UK where mad cow disease was at its worst and my family regularly ate and still eat beef. Your mom should be okay because its still kinda rare.
Stop feeding dead animals to other animals. Stop eating meat.
Good boy. Don't question that.
My grandpa got Mad Cow Disease while living in Ohio and died when my dad was a teenager. It can get you anywhere. Terrifying.
@@KallieMae memers trying not to say it :
CJD is terrifying - having done research into it for a project of mine and knowing someone who has the familial version, it's scary to think about. Even though the chance of it occurring is minimal for most people
Yeah, it is by far the scariest disease in my opinion. Even though its rare, its shocking how it can still easily happen. All it takes is to eat a peice of infected meat.
CJD (and of course other diseases in this class) are indeed very terrifying.
Just absolutely heartbreaking to witness someone's slow cognitive decline, from the span of just a few months to even a whole decade.
My heart goes out to everyone who has these horrid diseases, and I hope we can find a definitive cure someday.
CJD is quite fascinating but also scary, i don’t have anyone in my immediate relatives with CJD but it’s terrifying to think that i may get it one day.
i'll never look at cow meat the same way ever again
Hey aren't you the vCJD album man?
My uncle's friend died of CJD from tainted meat in a freak accident, it's super rare, but something I've definitely heard of.
Great. More to worry about
Sorry to hear of your uncle's friend's death.
@@MarcColten-us2pl Not if you switch to a fully plant based diet.
@@someguy2135 Something else horrible is lurking in the shadows,
@@MarcColten-us2pl I'll bite. What are you talking about?
Prion disease to me is like the neurological version of false vacuum decay, with the notable exception that it's real instead of hypothetical and it would happen inside your head instead of some unknown part of the universe unfathomably far away.
Excellent description
So when I was a kid, I first learned about prion diseases while playing Dead Island. I went on a research binge looking into how they work and the different outbreaks there have been, and for a long time I was terrified of them. It's such a scary thought, a mistake in our proteins that we can't control nor cure, but it's also great fuel for horror fiction so there's a small silver lining.
Remember those awful year’s I use to have nightmares I kept thinking I had it due to my stress
You should be terrified of them. Although that’s not to say you should spend every waking moment living in fear of them, but it’s very uncomfortable to think that one day you could just contract a prion disease and bam-you know you’re going to die very soon and very uncomfortably.
@@sigiligus Why be afraid of them? Besides not eating infected meat, there is nothing to be accomplished.
I am a medical doctor in indonesia, back at college my teacher told us about this disease, decades ago it was prevalent in papua island due to cultural practice of consuming the brains of deceased relatives in certain regions who believed that consuming the brains of their deceased relatives would allow them to inherit their wisdom and skills.
Thankfully the culture is no longer being practices.
Kuru!
kuru dieases… it’s a disgusting practice
@@eggrollsoup its disgutsing for some but for others esp a tribe with not much modern human contact its just part of their culture
Good lord what s4v4ges
My grandfather and I worked together at an auto body shop. We had a friend, well, really his friend, named Richard. We noticed over a few weeks of work he would come in and ask "What color did you want that car painted again?" or he'd trip on things, or lose tools.
One day, he came in with tears in his eyes and sat in the office. My grandfather and him sat and talked about how he was slipping. We called Richard's wife, had her come get him and take him home. About a week later, he gets a call from her. Bad news. Very bad news..
It was hard watching him become a shell of himself. Not long after, my grandfather followed him to the great paint booth in the sky.. R.i.p Richard Bernal and Gregory Engelsson.
How old was Richard?
Another prion related disease that scares the poo out of me is fatal insomnia. Something that pops into my mind whenever Im wide awake laying in bed and can't sleep. Now there is no logical reason for this to be the case but the mind is relentless sometimes.
I feel the same.
I think you also get extrem insomnia from rabies and that shit is also deadly and when you not get treated in 48 there is no cure.
Another thing to add to the "existential dread list" list. Right up there with brain eating amoebas.
Here's another one: there's a genetic condition (which happens to be another prion disease) called "fatal familial insomnia" that starts off as mild insomnia, but progressively gets worse and worse as symptoms like rapid weight loss, hallucinations, and even dementia start happening, until one day you just drop dead.
@@Arcterion NO
WE ARE NOT HAVING THIS DISCUSSION NOW
How about all the diseases that like, one person has?
@@jesseyu69420 I don't know them so I can't be scared of them... yet.
@@cavemann_ Rabies. 6 people have survived it without the vaccine. Basically, rabies has a 99.9% mortality rate.
Prion disease are so fascinating - they manage to be spontaneous, heritable, and contagious all at once.
Having lost my aunt to this disease last year i almost didnt watch this video.... im glad i did though. its quite scary how fast she went from happy and energetic to just sitting in a chair unable to communicate. i dont know if it whether knowing that she would die within a year made it easier or harder to deal with for me. i still wish i was able to visit her more in the last year, but it is what it is, cant change the past.
remember to show people your love and appreciation when you can one day they might not be here.
I am usually not huge into listening to sponsor spots. However this one does sound very interesting, and with large benefits for the researchers in the field.
I was actually annoyed that sponsorblock didn't kick in for the first few seconds lol
Yeah, having IA to do the heavy lifting is something I was expecting for years
The worst part of this disease is that it can happen randomly at any moment or any time without you knowing, and it can even appear when you are in perfect condition
But sametime you could be afraid of meteor coming through your roof at night
I’ve heard of this, it’s called “Kuru”. I watched it a long time ago. Story was, an indigenous group would cannabilize there dead tribesmen, and eat there brains, and the disease would spread from one person who ate the brain from the diseased infected person to the individual consuming them. Scared me ever since I heard it.
Kuru is one of the many diseases caused by prions!
CJD is the spontaneous/inherited form of kuru. Interestingly, some people within that group had a natural immunity to the disease!
Women and children were disproportionately affected by Kuru because they tended to eat the brain whereas men would eat muscles.
Sounds like the origin story for zombies in general.
It's not the same exact disease, prion diseases both are yes. But they are different
As someone who learned about CJD and it’s Progression from a project (someone mentioned it about his project) man it’s genuinely is scary how it just.. annihilates someone so fast.
Fast? The most terrifying thing about it is that it's so slow
@@georgeuferov1497 It kills over like the time period of a year or less, AD kills someone over the course of around 6-10 years.
@@georgeuferov1497 it's incubation period can be quite long, but once symptoms appear, the disease progresses quickly
Actually it's like if you removed ram from a working computer, with the only difference that computer will return normal just by putting the ram back
@@marcovaleriofranco9310 nervous system is a bit more than RAM
I try not to think about CJD too much because there’s nothing that can be done about it, but I do have that little nagging worry in the back of my mind about it. I fall into two "elevated risk" groups - I lived in the UK (eating plenty of beef) for a time back at the start of the 1990s, and I’m also a type 1 diabetic who used insulin derived from cows for more than a decade prior to the introduction of synthetic human insulin. My chances of having contracted CJD are very tiny, but I know it can appear seemingly out of nowhere even decades after the exposure to the prions occurred.
My father-in-law died from CJD. After watching him fade away the way he did, I'll never be the same. It was so humbling in how powerless everyone felt.
Within 5 seconds of starting the video I said “she’s Dutch!” 😂 such a recognizable accent, haha
literally the moment they start with "there's" xD
My mom was nurse on neurology, she said there was woman (63-64) that came walking and consiuous , that
woman was diagnosied with CJD then the progress rapidly worse. My mom was telling it was horifing because that woman came walking and after few months the woman cannot move. That woman very often starting randomly screaming, cannot move eyes and cannot rebember anything. Its very sad.
I see you adding Grusha and Iono into the video there! Something to cheer you up after seeing all the tragic stories 2:18
I thought I was the only one who noticed that
Pokemon reference???
@@cloverisfan818yuppp
CJD scares the shit out of me because I live in one of the epicentres of CJD and I work in a hospital and we always have patients with it
Where do you work?
The BSE/CJD outbreak in the 90's was pretty crazy. Did we even know it was caused by prions at the time, or was that outbreak the reason we discovered it?
It turned me vegetatian for a while (the outbreak)
That's how it was discovered to my knowledge. And how we discovered it's origins in cannibalism. Since the cows were being fed cow byproducts to help cut their feed. Also realizing how tough prions are to destroy.
In the UK it was known for a long while that some sort of pandemic was occuring.
But the UK government covered it up for about 10 years.
There is a good plainly different video about this.
@@koharumi1 you mean “plainly difficult” an excellent UA-cam channel that explains various disasters. I saw the one on BSE, I agree, it’s worth a watch.
The disease found in certain hunter gatherer tribes in New Guinea that practice cannibalism is called “kuru” and I remember learning about kuru in the mid-1980’s, prions were known by then (maybe not well understood) but that was before the BSE outbreak.
Also transmissible via blood donations, which is why the blood donation questionnaires used to ask about living in the UK during the period when the Mad Cow Disease scare hit. These days AFAIK they no longer ask about that, just whether you have CJD.
I remember when the BSE (AKA Mad Cow Disease) issue was first spotted; there was a lot of concern about possible prion infection, and beef exports from the UK were banned for a while.
They still ask about having taken growth hormone back when it was cadaver-derived. I'll probably never be able to donate. But my blood type isn't super valuable and I have no idea how my hormone situation would interact with blood donation anyway.
Dental procedures and certain optical exams. Runoff from infected fields. Excreted by birds like crows, etc., etc. There is much they are NOT telling you.
My condolences to anyone who has or lost a loved one to this disease ❤️
As much as I love origami, personally I think weaving would be a better metaphor for how proteins twist and knot into shape. Afterall, they are more like threads than like two dimensional planes.
Isn't folding done in 3D space?
@@smurfyday You fold TWO D surfaces in 3D spaces. A piece of paper is essentially TWO dimensional, by which I mean it has two main dimensions that define its geometry more than the third. In that way, it is like a PLANE (a 2D mathematical object). Planes exist in 3D space, but a plane is not 3D in itself, because it has only length and width. Paper has length length, width, and thickness, but again the thickness is negligible compared to the other TWO dimensions, so THAT'S why it is like a TWO-D PLANE.
Strings are essentially 1D, because they are defined most by a SINGLE dimension, ie length. Strings have thickness too (giving them a width and a height), but those features are negligible, so we can conceptualize them as a 1D mathematical object, ie a line. Lines can curve in a 2D plane, or even in 3D space, but they are fundamentally single dimensional objects.
Proteins are CHAINS of amino acids, meaning that their LENGTH is their primary dimension, while width and height are, in comparison, negligible. They are more like STRINGS than like paper or cloth. Thus, weaving or crocheting, something where you form KNOTS would be a better visual metaphor than origami.
BOTH the folding of paper and weaving of string is done in THREE-D space.Tthat doesn't fucking matter. It's a total non-sequitur. It's absolutely beside the fucking point. Have a spelled this out enough for you?
@@rdreher7380 Thanks for the detailed explanation. I'm sorry you're in a bad mental state
@@smurfyday You sound remarkably sincere. It's refreshing and disarming. Kudos. I'm sorry I'm in a bad mental state too.
@@rdreher7380 * hugs * I've been there. I admit it can seem simple, but I didn't see the analogy till you pointed it out
Hi, im from a small city in Chile, here we have around 20 family-groups that have the hereditary form of CJD. Around 20 people from one of these groups died last year
That must be difficult to live with! 😔
Weird but I gave you a thumbs up not because of anything yay but that is very interesting.
This is legit the scariest desease to me. By pure Chance one of your Prions folds the wrong way and boom, 2 years later your Brain is a freaking sponge, wtf
I have a family member who died from this. She was my grandma's cousin (grandma was adopted). During her last Christmas; she remarked that she felt off, she knew something was wrong. Less than half a year later, she was just....gone. It was scary to see. It was like someone took Alzheimer's and hit fast-forward.
I've been wondering, if once we figure out how the prion cause other proteins to misfold, would it be possible to medicate someone with a non-prion misfolded protein disease (e.g. cystic fibrosis) with a contagious healthy protein?
Anyone I asked dismissed or ignored the idea, but the yeast example tells me this might be possible after all
You just exposed one of the most cool ideas I have read about potential treatments. It's called interference. We still have to find out a prion that won't be toxic and can stop the other, toxic one. you're very clever!!! (I study these diseases)
@@di3486 That’s chill ngl
Well 12 years after losing my aunt to this i come accross this video and finally learn why it happened. Thank you for clearing this up for me even though it wasnt your intention.
Wow, this is terrifying! Great job!
Imagine being struck by lightning and living to only to immediately have a misfold resulting in a prion and death
A dear friend of mine's family has a genetic history of dying from this at an early age, it's terrifying for me to think that she has a 50/50 chance of dying at age 50 from this horrible thing.
@Charizard S if it’s genetic then maybe, if we can figure out which chromosome it’s on and then alter that DNA. Otherwise it seems to be due to chance, unfortunately
@Charizard S Unfortunately AFAIK there aren't any ways to prevent it.
This runs in my family. A variant called Fatal Familial Insomnia (FFI). My father died at 47 and my older brother at 48. I am next.
In early 2000's europe had a creutzeld jacobs scare because a couple of cases. I turned vegetarian for a while because of it. After the scare I went back to eating meat and after a few years went back to vegetarianism for ethical reasons and eventually turned vegan. But I never forgot that initial scare
Glad to see you are now vegan, despite having returned to meat eating for a while. First and foremost living vegan is the ethical choice, but there are many other advantages, including vastly reduced risk of contracting diseases like BSE.
@@jpe1 having a fear of contracting a disease like that is pure paranoia and a great example of confirmation bias. How many people die, or even contract a disease like this through foodborne pathways per year across the entire planet? It's just laughably rare, a person is probably more likely to die to autoerotic asphyxiation. There are a bunch of legitimate reasons to be vegan, but this disease is not one of them. (I am not vegan or vegetarian, animals are delicious.)
@@jpe1 Don't care. Unethically farmed chicken nugget go chomp chomp.
Our time on this planet is so limited, why the hell do you want to waste it on taking ethics into... the food chain?
@@rikuleinonen our time on this planet is indeed limited, so why would you want to waste it living your life poorly? Let’s live the best life possible! Is a life lived selfishly, looking only as far as what is most pleasurable in this moment, the way to live the best life possible? Or is life more fulfilling, richer and better lived, when you live it taking others into consideration?
@@adamh1228 I don’t know why your comment is shadow-banned, but I will respond regardless.
You are correct that vCJD is rare, to date only 178 people died of vCJD from eating contaminated beef (more have died from other food born illnesses) though that number would have been much higher if the British government had simply ignored the problem and not taken the drastic measures that they did. I wasn’t able to find reliable numbers on autoerotic asphyxiation (it gets lumped in with erotic (non-solo) asphyxiation, among other limitations in the data) but based on a rough estimate of 0.5 cases per million population per year in Western countries, that makes it roughly half as likely. But the important factor in both is that the individual has control of the circumstances, and can easily avoid being in a situation that could lead to premature death.
How is any of this confirmation bias? What bias is being confirmed?
Note that I didn’t advocate going vegan to avoid disease, I said that vegan is the ethical choice, that the reason to choose a vegan lifestyle is to minimize harm to animals; the other benefits, like reducing risk of disease, is bonus.
Your position that you choose to harm animals because they are delicious sounds as ridiculous to me as someone who says that African peoples should be slaves because they are intellectually inferior.
I've seen some videos on the topic in the past, but, in just over a minute, I learned far more about it than I did in those other, much longer videos. Of course, after the 1:15, mark, I continued and learned a bit more. This is what I expect from informational videos, but most channels take material that's good for less than five minutes, and extend it fifteen or more minutes...by babbling and repeating themselves. You've earned my sub.
I love it how even with such a dark topic the music is still cheerful. Topical.
I believe it was once cited as the top reason against cannibalism, or at least a part of research on an indigenous tribe.
The disease is called Kuro and I think is unique to some islands in Polynesia.
Kuru. And it was the Fore people.
@@galehunter2519 Are Fore people covered head to toe in Foreskin?
@@vice.nor.virtue Wtf 😂
@@kanjakan Yeah, bro. Never serve the fore people expired milk either. It can get a hell of a lot weirder. Trust me... 😦
My aunt died of this disease a while ago. Back then nobody had heard of it and thought it came from cannibalism.
Thank you MinuteEarth for giving me an existential crisis at 7am.
My microbiology professor in college mentioned that a form of CJD called Kuru occurred in a particular tribe in Papua New Guinea where cannibalism was common practice. Transmission was through ingestion of infected human neural tissue.
It's crazy how something so small can be so deadly just got to be careful of what you eat for the most part I guess
There's literally nothing that you as a consumer can do to spot this in the meat you eat. It's entirely up to the meat industry to catch this problem before it reaches the public, and this is a responsibility that is taken very seriously. In the 1990s one million head of cattle were slaughtered in the UK due to the public safety risk of _Mad Cow Disease_ - it was a massive news item at the time and it took a long time and a lot of precautions before faith was restored in the livestock industry.
Polonium-210 atoms and the alpha radiation released from it is smaller.
Prions are one of my favorites subjects to discuss, but also the diseases I am most terrified of
There is one other CJD type you didn't mention: Kuru. This is when CJD is in humans, and it was also passed on by eating (in this case, a funereal rite). When this funereal rite was finally discontinued, the disease went away.
i read that CJD and kuru were the same disease presenting in humans, just dofferent names, while the forms of CJD in animals were called scrapie (sheep) or CWD (deer). Kuru was translated from the native language to something like the laughing disease cause of the shaking and grimacing CJD induces in its victims
Read that in a book awhile tho so I could’ve misunderstood, I’m not an expert at all
@@kida6262 Yes, the other name for Kuru was Laughing Sickness. And if they aren't the same disease, they are very closely related.l
@@kida6262 The laughing death
Lizah, I love your accent. And I love your writing and animation! I hope to hear more of you!
I loved all of the illustration references in these videos!
I learned about prions when I looked up fatal insomnia. These diseases are so terrifying indeed. I feel for any person who gets these diseases.
I remember reading about how prion dieseases work and being baffled, because that's like 3rd way of reproduction for a dangerous agent, even more basic than a virus yet really similar. There are bacteria, viria and prions. Each one gets simpler but they all essentially are about getting a copy of itself.
Maybe it relates to our far far ancestor cells dividing generation through generation to multicellular to an even more complex structures to survive disasters and the harsh environmental changes. At that time, perhaps what was the most important is the passing of information generation through generation for survival. Our existence is thanks to those cells.
Excellent presentation, thank you!
My grandma recently died from CJD. We only knew something serious (more than a concussion) was up two weeks before she died.
My next door neighbor actually died from this. Total shock when it happened.
Always nice to have fellow Dutch people do well on UA-cam :)
Wonderfully explained.👏👏
Rare, like getting hit by lighting. That doesn't comfort me. I HAVE been hit by lightning.
I remember learning about proteein folding, The term Chaperonies describes a protein, that helps other proteins being folded the right way. Alzheimers disease is also suggested to be formed by clumpsed, deformed proteins, that can not be degraded. The proteins structures can be investigated by roentgen structure analysis. This method is super nerdy and awesome but very hard to be performed. It wold be great to make a video about that!
As far as I can tell, the latest drugs for cystic fibrosis somehow chaperone proteins that are destined to misfold because of DNA mutations, and encourage them to fold correctly. (I think?)
Anyway, it seems like magic to my feeble mind, and would make an interesting video.
At 2:30 all the people watching that have been struck by lightning and survived started sweating profusely.
If only Grusha and Iono from Pokemon had the power to fix prions
fascinating phenomenon and very nicely explained in this video. thanks
new fear unlocked
Congrats for minute earth again and again for giving me more anxiety. I'm joking, I love your work and what you do for us.
The videos you guys make are really good. Wish i had some spare money to support you guys...
Great job, Lizah!
i remember the 90's scandal . This disease is the reminder cannibalism is a bad thing , among humans those who practice it suffer of a high rate of CJD , from the late 70´s they feed the cattle with a mix that contain flour made of disposable cow parts from slaughters . The worse part is that CJD take up to 20 years to start showing is effects
I recently did an MRI on a patient with this. I washed my hands well afterward.
It is in nasal passages and saliva. Did you wear a gas mask?
@@litsci4690 it is not airborne. The odds of getting it are tiny tiny tiny. But consequences are big ofc.
@@cade8986 WTF do you know? Patient sneezes. You inhale. DUH. Patient touches nose or mouth and touches equipment. Still infects 15 years later. Read some actual scientific articles. Don't just ignorantly repeat whatever you're told. Wash your hands? What a joke! An autoclave won't even remove it from surgical equipment!
prion related diseases give me panic attacks since i found out about them, i hope that in the future medicine will find a cure or preventive treatment for it
I associate the Dutch accent combined
with fluent English with expert knowledge thanks to people like these
Fatal familial insomnia is terrifying
FFI killed my father & older brother and I have the gene that carries it. Living on borrowed time.
I thought the title said "How Prisons Destroy Your Brain" at first
Same, I was extremely confused :D
Gotta be the next video... I hope
Also a valid subject, but maybe not for this channel.
Great explanation!
Ooh, deepmind AlphaFold is so cool! Two Minute Papers has got a great video on it!
My best friend, his brother and father died of CJD a couple years ago. From first symptoms to death it was 6 months. It was not a good death either. Your brain malfunctions worse and worse until you die. You stop being you long before the end. I spoke to a hospice nurse who was there. I mentioned how rare CJD was and she said it wasn't as rare as people thought and there were hot spots where numbers of people had died, it just didn't make the news. After all of that I understand why this disease scares doctors.
im probably gonna get it someday knowing my luck
@@thefogitself It can lay dormant for a decade before symptoms show.
@@matthewdavies2057 i know
@@matthewdavies2057 but is there any way to know if you have a prion disease before it starts
@@thefogitself There wasn't a few years ago. The center for study of that, I was going to call it a disease, malfunction is in San Francisco. Or it was.
That is THE most terrifying thing I have ever heard and I wish I could've remained in bliss
It seems like PROTACs which are specific for the mis-folded prion, or molecular glues which stabilize the correctly folded form of the prion have huge potential for treatments
CJD (and all other prion diseases) is one of the only two diseases that are able to keep me up at night in fear of them
I'm guessing the other is either rabies or Alzheimer's
More Lizah please! Her voice is GREAT!
The misfolding is because the proper working protiens are in an energetically metastable state. When exposed to a misfolded one, the electric field pulls the hydrogen bonds enough to have the prion dimain region collapse into the energetically favorable condition. One possible way to treat the disease would be to use a deuterated amino acid that has stronger hydrogen bonding preventing the field disruption. This would require a delicate balance though as too much would cause the very effect you want to avoid ❤
CWD is another fear of mine, some think a man may have developed CJD from eating an infected deer. no part of a deer with CWD is safe, although the prions only live in the central nervous system
I wish people showed this much fascination for other diseases that are equally fatal but more preventable and more commonly risked, like the "brain-eating" amoeba naegleria fowleri (summer is amoeba season, wear nose clips when playing in fresh or brackish water and just generally don't force nonsterile water up your nose!)
i usually clean my nose by dipping my nose into tap water and sucking it up so that it goes into my throught through my nose cavern. am i not supposed to do that?
I agree. You also see people joking around with dangerous things and not taking them seriously. Everytime I see a video of someone interacting with animals like a raccoon or a bat I get uncomfortable, as I have an insane fear of rabies. Just kinda ran with that example as it's preventable with vaccines
Prions seem almost like the strange matter of the cellular level
Hello Lizah! Great video! Also, that's a Dutch accent if I ever heard one! 😄 Leuk dat minute earth/Yikes is so international.
this video gave me every symptom and form of this
I was watching a couple streamers play Dayz and they were eating steaks cut from the one guy's previously killed character. I was like hmm I wonder if they have prion diseases in this game. Later in the playthrough their characters started going crazy, so I think it might have actually been factored in lol.
nah, that’s just insanity. it doesn’t have any other side effects aside from making your character just laugh like a maniac occasionally, which attracts/scares basically anything in your general area and is impossible to get rid of afterwards. well, i guess when put like that, it sorta sounds like it huh…
“But don’t worry! CJD is very rare!!” Comments filled with a bunch of first hand anecdotes 💀
Remember that while there can be many people who simply lie.
But people with own hand experience are more likely to randomly find this video because algorithms. Or even search it.
there's someone in the comment section who said that CJD isn't as rare as people make it out to be, and I read an article abt how CJD is rare but not 1 in a million chance, it's more common than that.
Thanks for your Sharing
hopefully we'll find a molecule that can bind to Prions and not the correctly folded proteins, since the shape varies, so should the interaction, the difficulty is finding that molecule and see whether it's safe for humans and cattle.
Two minutes in, I just realized that the title does _not_ say "prisons". *Now* it makes sense! 🤯
Why wouldnt RNAi be used to cure prion diseases? Denature the proteins with hairpin loop tech, you could even target the specific misfold