New Brunswick monitoring more than 40 cases of unknown neurological disease(cbc.ca)
cbc.ca
New Brunswick monitoring more than 40 cases of unknown neurological disease
https://www.cbc.ca/news/canada/new-brunswick/mad-cow-disease-public-health-1.5953478
130 comments
Holy shit it was chilling to read those articles, knowing full well what they represented.
“It would be a nightmare for all of us.”
I bet that doctor would call that an understatement when asked now, or even 2 months later.
I bet that doctor would call that an understatement when asked now, or even 2 months later.
> SARS originated in China and killed more than 800 people worldwide in 2002 and 2003
Only 800, yet it felt like such a big deal back then...
Only 800, yet it felt like such a big deal back then...
The mortality rate is what terrified people about SARS. It was a big deal. 50% for people 65 and older. 15% in the general population. This incidentally made it far less reproductive.
> This incidentally made it far less reproductive.
Unfortunately not. SARS-1 reproduced slowly (long onset), wasn't all that contagious, and was quite deadly.
These are independent variables. Smallpox had a high R0 and was quite deadly.
Unfortunately not. SARS-1 reproduced slowly (long onset), wasn't all that contagious, and was quite deadly.
These are independent variables. Smallpox had a high R0 and was quite deadly.
And most importantly, was contagious before the beginning of the symptoms, which always included fever so it was pretty easy to scan people with thermometer to catch the contagious ones.
> And the fact that SARS was only contagious several days after the beginning of the symptoms is the reason why we have been able to control it […] and had contagiousness begun far earlier after the beginning of the symptoms or even before them like for the flu, the outbreak would have exploded and we wouldn't have been able to halt it. [1]
[1]: quick translation from French from https://www.college-de-france.fr/site/arnaud-fontanet/course... (24'05)
> And the fact that SARS was only contagious several days after the beginning of the symptoms is the reason why we have been able to control it […] and had contagiousness begun far earlier after the beginning of the symptoms or even before them like for the flu, the outbreak would have exploded and we wouldn't have been able to halt it. [1]
[1]: quick translation from French from https://www.college-de-france.fr/site/arnaud-fontanet/course... (24'05)
In the modern world, deadliness decreases transmissibility (Re, not R0), because people make organized efforts to contain deadly diseases.
Covid's death rate was low enough that a good fraction of people thought we should just take it on the chin.
By contrast, Smallpox was eradicated because it was so deadly, meaning we pushed its Re below 1.
Covid's death rate was low enough that a good fraction of people thought we should just take it on the chin.
By contrast, Smallpox was eradicated because it was so deadly, meaning we pushed its Re below 1.
Yeah that's what I was getting at. SARS-COV-2 seems to have hit this sweet spot of being deadly enough to eclipse the death toll of similar viruses like influenza, but not deadly enough for people to take it as an existential threat. I'll take GP's word that it's also less transmissable, I was just saying that all things being equal, if SARS had a lower mortality rate, there's a chance it wouldn't have been dealt with in the same way.
Ah, fair play. I thought you were purveying the common misconception that a virus which kills its host is less effective at finding new hosts than one which doesn't, but this is unfortunately not the case.
Alas, your actual theory doesn't really hold up either. It's tough to remember the mood thirteen months ago, but China was under a real lockdown, we were seeing videos of people passing out in the street (funny, that doesn't seem to have kept happening?) and apartment building being welded shut, there were reports circulating of nitrogen-rich plumes of smoke in Wuhan which might be mass incineration of bodies: people were really scared. America even closed the border to China, against the explicit policy of WHO, but didn't shut down travel from Europe fast enough; also, the current consensus is that SARS-2 was circulating in the US by mid-December 2019 at the latest, so it's unclear how much benefit a total travel ban would have provided.
But, no: SARS-1 doesn't become highly infectious until after symptoms set in, that was what saved us last time. It does seem to be true that countries which experienced the SARS epidemic first-hand developed what we might call institutional antibodies, and were quick to impose various controls (especially contact tracing) which were more effective at controlling COVID-19.
But COVID-19 has a long period of presymptomatic infectivity, it sheds for almost a week during which the patient feels fine. If SARS presented with that etiology, there is a good chance that it would have swept the planet, killing, potentially, hundreds of millions of people.
Just dumb luck.
Alas, your actual theory doesn't really hold up either. It's tough to remember the mood thirteen months ago, but China was under a real lockdown, we were seeing videos of people passing out in the street (funny, that doesn't seem to have kept happening?) and apartment building being welded shut, there were reports circulating of nitrogen-rich plumes of smoke in Wuhan which might be mass incineration of bodies: people were really scared. America even closed the border to China, against the explicit policy of WHO, but didn't shut down travel from Europe fast enough; also, the current consensus is that SARS-2 was circulating in the US by mid-December 2019 at the latest, so it's unclear how much benefit a total travel ban would have provided.
But, no: SARS-1 doesn't become highly infectious until after symptoms set in, that was what saved us last time. It does seem to be true that countries which experienced the SARS epidemic first-hand developed what we might call institutional antibodies, and were quick to impose various controls (especially contact tracing) which were more effective at controlling COVID-19.
But COVID-19 has a long period of presymptomatic infectivity, it sheds for almost a week during which the patient feels fine. If SARS presented with that etiology, there is a good chance that it would have swept the planet, killing, potentially, hundreds of millions of people.
Just dumb luck.
> It does seem to be true that countries which experienced the SARS epidemic first-hand developed what we might call institutional antibodies, and were quick to impose various controls (especially contact tracing) which were more effective at controlling COVID-19.
Ironically in some ways it had the opposite effect in Canada. We remembered how hard-hit Toronto's tourism industry was as a result of travel advisories from SARS-1 and so were initially supportive of China+WHO's argument against banning travel. But like you said, it's really hard to see this not being a pandemic either way, it might've just bought a bit of valuable time.
Ironically in some ways it had the opposite effect in Canada. We remembered how hard-hit Toronto's tourism industry was as a result of travel advisories from SARS-1 and so were initially supportive of China+WHO's argument against banning travel. But like you said, it's really hard to see this not being a pandemic either way, it might've just bought a bit of valuable time.
>A first case was diagnosed in 2015, according to the memo. Three years later, in 2019, 11 additional cases were discovered, with 24 more cases discovered in 2020 and another six cases in 2021. Five people have died.
So not 40 cases all at once in case anyone was worried.
So not 40 cases all at once in case anyone was worried.
That would probably be less worrying to me, would indicate whatever got them is now gone, right? Rather than it is something that has stuck around to poison more people within 5 years
If it's a prion disease, they sometimes take years or possibly decades to develop.
The big question is how they were all infected. Is it in the food supply (meat, milk)? Was it tainted blood donations?
42 people is a lot of people... The vCJD outbreak in the 90s (mad cow) ended up with less than 250 cases total.
The big question is how they were all infected. Is it in the food supply (meat, milk)? Was it tainted blood donations?
42 people is a lot of people... The vCJD outbreak in the 90s (mad cow) ended up with less than 250 cases total.
We weren't cleanly segregating brain/spine tissue during meat processing in Canada until fairly recently. Given that CJD is only diagnosed by autopsy, I assume some fraction of dementia/Alzheimer's cases are actually prion diseases.
And since we have no treatment, assigning liability is impossible, and it would destroy the beef industry, there's not much enthusiasm for learning more. The premier of Alberta was very clear: shoot, shovel, and shut up.
And since we have no treatment, assigning liability is impossible, and it would destroy the beef industry, there's not much enthusiasm for learning more. The premier of Alberta was very clear: shoot, shovel, and shut up.
Whilst the amyloid plaques in Alzheimer’s could be considered a type of protein disorder with similarities to prion diseases in that a protein is aggregating in clusters that the body is unable to clear and break down (difference being, it isn’t infectious in origin and a amyloid beta protein isn’t being changed by a misfolded protein of its own type) I don’t think there is any evidence to support your assumption that some fraction of dementia or Alzheimer’s presumptive disgnostic patients (Alzheimer’s also being largely a diagnosis enabled by autopsy, although there are scans that can show the amyloid plaques) because the diseases have quite different symptoms and courses.
Whilst that can’t fully rule out unknown prion diseases out there, generally we would expect such a condition to manifest in a much broader section of the population than just ‘the elderly’ and when young people develop significant neurological symptoms there’s a huge amount of diagnostic effort that is put in to determining what is going on, because, you know, it might be a prion disease and generally it shouldn’t happen unless there is a genetic determinant or another cause, such as smoking adulterated drugs which was found to cause Parkinson’s in the 80’s [0], or in fact vCJD
[0] https://www.cdc.gov/mmwr/preview/mmwrhtml/00000360.htm
Whilst that can’t fully rule out unknown prion diseases out there, generally we would expect such a condition to manifest in a much broader section of the population than just ‘the elderly’ and when young people develop significant neurological symptoms there’s a huge amount of diagnostic effort that is put in to determining what is going on, because, you know, it might be a prion disease and generally it shouldn’t happen unless there is a genetic determinant or another cause, such as smoking adulterated drugs which was found to cause Parkinson’s in the 80’s [0], or in fact vCJD
[0] https://www.cdc.gov/mmwr/preview/mmwrhtml/00000360.htm
You're right that young people are investigated, and we don't see broad infection. I was thinking of causes for geriatric CJD, rather than vCJD. I'm skeptical of claims that geriatric declines are fully investigated. But the UK spike from contaminated food was very clear, and we don't see anything similar. Unless there's some low-level exposure the body can resist and a weakening blood-brain barrier/stroke/etc allows infection. But that's just speculation. Cheers.
Also, the prions themselves are not easily deactivated, and can linger in the environment, in an infectious state, for decades.
In my area we have a prion disease called CWD in the whitetail deer population. There's basically no way to destroy them in the wild. You have to bleach or burn the wilderness.
They've had some suspicions that the prion has infected humans, although I haven't read any updates to that in a couple of years.
Many locals still don't get their kills checked, even though it's free. They just eat them. A lot of the logic is that it's been here a long time and they've probably already eaten it.
Anyway, prions are the scariest thing I've heard of in a long time, because they don't even have an agenda.
They've had some suspicions that the prion has infected humans, although I haven't read any updates to that in a couple of years.
Many locals still don't get their kills checked, even though it's free. They just eat them. A lot of the logic is that it's been here a long time and they've probably already eaten it.
Anyway, prions are the scariest thing I've heard of in a long time, because they don't even have an agenda.
Wow didn't realize how big that was in the Rockies, like Alberta Canada or Colorado. Both places I've lived and hunted in...
Jeez don't even suggest that. If that shit jumped to humans we are fucked.
I'd rather not imagine how that would look like in comparison to covid
I'd rather not imagine how that would look like in comparison to covid
It already jumped to humans. Mad cow disease infects humans as well.
The difference in infection levels is that mad cow disease is transmitted through ingestion, so the usual avenue for human-to-human transmission is cannibalism (see e.g. the outbreak of Kuru that resulted from ritual funerary cannibalism), which is rare. Animal-to-animal cannibalism in animal feed is more common.
The difference in infection levels is that mad cow disease is transmitted through ingestion, so the usual avenue for human-to-human transmission is cannibalism (see e.g. the outbreak of Kuru that resulted from ritual funerary cannibalism), which is rare. Animal-to-animal cannibalism in animal feed is more common.
Theoretically, could someone weaponize prions? Perhaps there'd be no point if the same tactics would be more effective with a typical deadly pathogen (ebola or something), but in theory could a malicious actor take human/animal brains confirmed to have a prion disease and then mash up and aerosolize the brain matter? Or would it not be possible to get infected through inhalation?
I wonder if fully cooked meat can also transmit this disease? I know its big deal eating tainted meat as all hunters are encouraged to have the meat tested before consumption. there have been alot of deer that have been shown to have this disease.
Fully cooked meat can also transmit prion-based diseases, because cooking isn't hot enough to destroy the prions. IIRC, you need 1500 degrees (Fahrenheit, I think). Your stove or grill isn't going to get you there, no matter how thoroughly you cook the meat.
Wouldn’t the deactivation temp depend on the prion?
Prions are why we use more disposable tools for surgeries instead autoclaving. Autoclaves aren’t hot enough to destroy the proteins.
Sure, but most prion diseases are confirmed to be resultant from various misfolding of a single protein https://en.wikipedia.org/wiki/PRNP
I have not eaten a piece of Beef since the 90's when there was that madcow scare. I was also very particular about trying to eat meat that was cooked on the same surface as beef. Now I am not as neurotic.
I was also the kid that stayed awake at night worrying about Ebola when there was an outbreak in Zaire in 1995 I think it was.
I was also the kid that stayed awake at night worrying about Ebola when there was an outbreak in Zaire in 1995 I think it was.
I stopped eating beef for a long time after that, then stayed away from ground beef for a lot longer. These days I eat gourmet burgers from time to time.
The carbon footprint of beef is a disaster anyway. There are plenty of other reasons to stay away.
The carbon footprint of beef is a disaster anyway. There are plenty of other reasons to stay away.
Genuinely curious, based on how you described yourself, if you’ll be getting the COVID-19 vaccine?
I am not as paranoid an adult but CJD and Ebola scare the shit of me still.
The survival rate of Covid-19 is higher than both Ebola and anything prion based so Covid-19 doesn't scare me as much.
I will get the shot. Most of my family has already(medical profession). If my parents get Covid-19 they are as good as dead so I will be getting it for their sake also.
I will get the shot. Most of my family has already(medical profession). If my parents get Covid-19 they are as good as dead so I will be getting it for their sake also.
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So, prions are like, a level of "life" below even viruses. They're not alive (and neither are viruses technically). They're essentially just misfolded proteins that when a cell attempts to break the protein apart and use it, it causes a problem in the enzymes that break the protein and forces them to replicate that protein.
Mad cow disease was (is?) a prion disease. Since they're just proteins and not living creatures, even heating them doesn't kill them. They can't infect you like a virus or bacteria can, they basically all do the same thing, cause brain deterioration. They can't mutate to become more virulent, and they are spread through food consumption and usually caused by cannibalism. Whether a prion can damage cells in an animals brain depends on its DNA sequence and the enzymes their cells create to break apart certain proteins. We currently don't know if chronic wasting disease can hurt humans, and there are tons of prions all over the place all the time and you ingest them and most of them don't do anything bad to you. So there's no telling with CWD yet.
Mad cow disease was (is?) a prion disease. Since they're just proteins and not living creatures, even heating them doesn't kill them. They can't infect you like a virus or bacteria can, they basically all do the same thing, cause brain deterioration. They can't mutate to become more virulent, and they are spread through food consumption and usually caused by cannibalism. Whether a prion can damage cells in an animals brain depends on its DNA sequence and the enzymes their cells create to break apart certain proteins. We currently don't know if chronic wasting disease can hurt humans, and there are tons of prions all over the place all the time and you ingest them and most of them don't do anything bad to you. So there's no telling with CWD yet.
One of the problems with prions is they tend to be very stable and stick around in the environment for a long time, even resiting heat, cold, and persisting for years in soil. To add on to that, when active in a human being, they can take a very long time to get to a point where symptoms emerge.
Or it just takes a variable amount of time for symptoms to show up. (Wikipedia says kuro which is a prion disease can take up to 50 years! Although maybe thats related to immunity which seems irrelavent here)
Didn't think about that, fair point
> So not 40 cases all at once in case anyone was worried
Yep, not connected to the mRNA vaccines. :)
Yep, not connected to the mRNA vaccines. :)
Should be interesting to see how long it takes to figure out how it's being transmitted. What all those 40 people had in common...some lake, or medical procedure, etc, that they all had. Assuming it is a prion disease...
Apparently prions are pretty hard to kill off, the sterilization procedures are pretty long. https://www.cdc.gov/prions/cjd/infection-control.html
Apparently prions are pretty hard to kill off, the sterilization procedures are pretty long. https://www.cdc.gov/prions/cjd/infection-control.html
Yeah, I'm not an expert but I've heard experts discuss it as though they're almost impossible to sterilize. As a layman, I imagine this has something to do with the fact that your task is essentially to break down a molecule (a protein) instead of a structure of molecules (a virus or bacteria particle). Heat may not be very effective and the likelihood that you eliminate all the prions to the point that they'd have no effect is lower.
Also not a chemist, but you’ve made me notice my confusion:
Aren’t proteins generally easy to denature? Isn’t that what most cooking does? And what alcohol does?
Aren’t proteins generally easy to denature? Isn’t that what most cooking does? And what alcohol does?
OK, so I worked seven years in this field. Prions (and amyloid diseases in general, like alzheimer's, diabetes, and a ton of diseases you probably haven't heard of [0]) happen when a "normally folded protein" denatures into a flat ribbon [1]. These ribbons become a sheet when you get many of them side-by-side, and this it turns out is an extremely stable configuration. Also, it's autocatalytic (exponential growth), because these sheets will fragment and recruit well-folded proteins (that are of the same molecule) to become ribbons at their ends, extending and growing the sheet.
So, yes, it is protein denaturation. But it's a very specific denaturation endpoint that not all proteins are capable of, and it's such a favored endpoint that even heating won't quantitatively destroy it (and heating could make it worse by fragmenting it into a bunch of smaller sheets).
[0] Incidentally, this denaturation is probably also used by cells in a productive fashion, it's pretty likely IMO that your melanin cells use these sheets to template melanin formation and sequester away the melanin molecule (the melanin precursor is highly toxic). I would link to the paper, but I think all of the data have been essentially cherry picked from shittily-done experiments, even though I think their conclusion is correct -- I have personally repeated the experiments "correctly" and reached the same endpoints in one shot without cherry picking (unpublished for my experiment, also n == 1) -- so I don't want to draw more unhealthy attention to the paper until someone else does the experiment "correctly".
[1] how does this happen? It can happen either due to a mutation that destabilizes the parent, well-folded protein, or, just by bad luck / time. Mutation examples: Fatal Familial Insomnia, non-variant CJD. Bad luck/Time example, insulin in a bottle in a fridge will turn into white flecks (these are sheets). The time at which this happens is highly variable (luck). You can accelerate this by agitating the bottle, and creating an air-water surface that catalyzes the denaturation of insulin. Also note, that insulin is not the protein in sheets in diabetes :biology trollface: (the insulin situation is strictly in vitro, and pH-dependent, as far as we can tell)
So, yes, it is protein denaturation. But it's a very specific denaturation endpoint that not all proteins are capable of, and it's such a favored endpoint that even heating won't quantitatively destroy it (and heating could make it worse by fragmenting it into a bunch of smaller sheets).
[0] Incidentally, this denaturation is probably also used by cells in a productive fashion, it's pretty likely IMO that your melanin cells use these sheets to template melanin formation and sequester away the melanin molecule (the melanin precursor is highly toxic). I would link to the paper, but I think all of the data have been essentially cherry picked from shittily-done experiments, even though I think their conclusion is correct -- I have personally repeated the experiments "correctly" and reached the same endpoints in one shot without cherry picking (unpublished for my experiment, also n == 1) -- so I don't want to draw more unhealthy attention to the paper until someone else does the experiment "correctly".
[1] how does this happen? It can happen either due to a mutation that destabilizes the parent, well-folded protein, or, just by bad luck / time. Mutation examples: Fatal Familial Insomnia, non-variant CJD. Bad luck/Time example, insulin in a bottle in a fridge will turn into white flecks (these are sheets). The time at which this happens is highly variable (luck). You can accelerate this by agitating the bottle, and creating an air-water surface that catalyzes the denaturation of insulin. Also note, that insulin is not the protein in sheets in diabetes :biology trollface: (the insulin situation is strictly in vitro, and pH-dependent, as far as we can tell)
I half expect a very scary Black Mirror episode where a militant vegan faction devises a way to spread prions throughout the meat supply chain on a timed release so it hits everywhere at once. Only for the blowback to somehow far exceed their worst dystopian nightmares.
Is there a way to somehow pack chemical or electrical energy into these highly favorable stable endpoint configurations, and release it?
Is there a way to somehow pack chemical or electrical energy into these highly favorable stable endpoint configurations, and release it?
It wouldn't be a black mirror episode unless these militants were able to record the memory of each of the animals before they were slaughtered, record that into the prions to have people experience the entire lifetime of an animal raised for slaughter with every bite... before succumbing to vCJD, of course.
The producers need to add you to the writing staff, brilliant twist. Prions are scary grey goo-reminding nastiness. I've sometimes wondered if they aren't the answer to the Fermi Paradox.
Sounds a bit like the plot of the movie _12 Monkeys_ (a very good movie).
Probably a very ignorant question, but don't protease enzymes break proteins down to the amino acids? Suppose my lab bench is infected with prions or flat protein sheets or something. If I spray it over with a bunch of protease, would I be able to "disinfect" it?
Great question. I'm going to answer in three stages.
1. Theoretically speaking, yes. And there's probably a biological protease that does it in the the body. Last I checked, it's still unidentified; my money is on Insulin-Degrading Enzyme.
2. It's more complicated than that. If you just buy an off the shelf protease, it probably won't work, because those are used to cutting up floppy bits of peptide sticking out of a protein, so to get there you would need to pull the sheet apart ribbon by ribbon first to be effective.
3. Given enough time, though, the ribbons ARE in equilibrium between being on/off of the ends of the sheet, so a protease could randomly catch the ribbon at the right time. But the equilibrium is so low, that your protease would probably self-digest faster than it takes apart the flat protein sheet.
1. Theoretically speaking, yes. And there's probably a biological protease that does it in the the body. Last I checked, it's still unidentified; my money is on Insulin-Degrading Enzyme.
2. It's more complicated than that. If you just buy an off the shelf protease, it probably won't work, because those are used to cutting up floppy bits of peptide sticking out of a protein, so to get there you would need to pull the sheet apart ribbon by ribbon first to be effective.
3. Given enough time, though, the ribbons ARE in equilibrium between being on/off of the ends of the sheet, so a protease could randomly catch the ribbon at the right time. But the equilibrium is so low, that your protease would probably self-digest faster than it takes apart the flat protein sheet.
Thanks for the excellent explanation! I feel like I understand, despite my total prior knowledge being merely highschool chemistry/biology and some YouTube videos :)
Thanks for sharing all this Isaac, is incredibly interesting to read!
If you don't mind: what's the most likely source of this new disease? What's the worst case scenario, and how likely is it to play out?
If you don't mind: what's the most likely source of this new disease? What's the worst case scenario, and how likely is it to play out?
When something is so localized like that I immediately wonder if it isn't a genetic susceptibility. I still suspect that high death rates of covid in northern italy we'll find out was some hereditary susceptibility, especially since the elderly in those communities are less cosmopolitan (less genetic admixing)
Aside from that I think it's irresponsible for me to comment much on it since there is so little information in OP.
Aside from that I think it's irresponsible for me to comment much on it since there is so little information in OP.
Understood, thank you!
Comments like this are why I love HN.
So denaturing a protein generally involves interfering with the noncovalent interactions (e.g. hydrogen bonds, attractions between internal charges) that allow it to fold into the right functional shape. Often times, these proteins folded under conditions that carefully facilitated their coming out the right way, so simply removing the denaturing stimulus (e.g. allowing them to cool) won't result in a reversion to their former configuration. This is what you see, for example, in cooking, where there's a discrete change that doesn't revert.
Prions are generally much more stable and can revert back to dangerous form once denaturing conditions change back. It takes a lot more to denature them irreversibly than it does most proteins. This stability is part of what makes them so dangerous and difficult to remove.
Prions are generally much more stable and can revert back to dangerous form once denaturing conditions change back. It takes a lot more to denature them irreversibly than it does most proteins. This stability is part of what makes them so dangerous and difficult to remove.
> Prions are generally much more stable and can revert back to dangerous form once denaturing conditions change back.
Indeed I'd imagine that almost definitionally prions must be difficult to denature. Since they are non-living and therefore have no way of "seeking out food," durability is the only way they can compete for reproduction. That is they can only become infectious if their resistance to denaturing was sufficiently high to resist environmental damage until their next target comes around.
Indeed I'd imagine that almost definitionally prions must be difficult to denature. Since they are non-living and therefore have no way of "seeking out food," durability is the only way they can compete for reproduction. That is they can only become infectious if their resistance to denaturing was sufficiently high to resist environmental damage until their next target comes around.
Yeah, your logic is pretty good here. Prions are a super interesting emergent phenomenon of a complex system. In a weird way, like cancer or viruses, they're just an inevitability of having enough of certain materials interacting for long enough time. Proteins misfold in just such a way that allows them to induce the same type of misfolding in other proteins, leading to propagation. It's less a definitional thing, and more a matter of the fact that something more stable will persist longer, encounter more opportunities to propagate and preserve its form. That said, surviving harsh environments isn't an entirely necessary condition, as not all prion generation requires transmission. Unfortunately, if you have the wrong gene(s) broken, you're at risk of just generating them yourself. That said, transmission is significant, and since it involves needing to be able to survive a harsh outside environment with no propagation between hosts, extreme durability is a definite boon, as you suggest.
It’s unusual to discuss a non-living chemical in terms of reproductive fitness, but I guess the word fits…
There is currently no consensus on what constitutes a living thing. Some include viruses, some not. Most do not include prions, but prions do feature many of living things (eg, they evolve, adapt to their environment, replicate, etc).
Another commenter pointed out that the general idea of denaturing a protein is interfering with noncovalent interactions, generally by interrupting the structure that the complete sequence takes after the folding process. These structures give rise to the classic shapes that proteins take, the loops and sheets and clumps of atoms you see in renderings. The other commenter mentioned cooking, and this process is the same that happens when you cook an egg, the proteins in the egg white are denatured and turn into amorphous strands of protein.
However there is a difference between a denaturing process and a lysing (breaking) process, meaning that the actual covalent, chemical bonds between the component amino acids that make up a protein are broken. This bond is called the peptide bond, and they are extremely stable, and to break them without enzymes requires some crazy conditions like strong acids or bases and high heat and long periods of time. There is a class of enzymes called proteases that catalyze and speed up this breaking-down process, which is what cells use to digest and break down proteins.
However there is a difference between a denaturing process and a lysing (breaking) process, meaning that the actual covalent, chemical bonds between the component amino acids that make up a protein are broken. This bond is called the peptide bond, and they are extremely stable, and to break them without enzymes requires some crazy conditions like strong acids or bases and high heat and long periods of time. There is a class of enzymes called proteases that catalyze and speed up this breaking-down process, which is what cells use to digest and break down proteins.
Here's an article on it:
https://www.scientificamerican.com/article/surgical-exposure...
https://www.scientificamerican.com/article/surgical-exposure...
No idea, honestly. As I mentioned, I'm not an expert.
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An interesting quote from the wikipedia article on prions:
"In 2015, researchers at The University of Texas Health Science Center at Houston found that plants can be a vector for prions. When researchers fed hamsters grass that grew on ground where a deer that died with chronic wasting disease (CWD) was buried, the hamsters became ill with CWD, suggesting that prions can bind to plants, which then take them up into the leaf and stem structure, where they can be eaten by herbivores, thus completing the cycle. It is thus possible that there is a progressively accumulating number of prions in the environment."
Dark dreams of a planet's biosphere irreversibly becoming utterly hostile to organisms that use neurons to function, through accumulation of immortal zombie nanoparticles that convert more and more proteins to their side.
"In 2015, researchers at The University of Texas Health Science Center at Houston found that plants can be a vector for prions. When researchers fed hamsters grass that grew on ground where a deer that died with chronic wasting disease (CWD) was buried, the hamsters became ill with CWD, suggesting that prions can bind to plants, which then take them up into the leaf and stem structure, where they can be eaten by herbivores, thus completing the cycle. It is thus possible that there is a progressively accumulating number of prions in the environment."
Dark dreams of a planet's biosphere irreversibly becoming utterly hostile to organisms that use neurons to function, through accumulation of immortal zombie nanoparticles that convert more and more proteins to their side.
Moose hunting seems quite popular in NB. Moose are also susceptible to CWD.
The moose population there has increased nearly 10x since 1960. In the 2020 hunting season, 3900 moose were taken. That is more than the entire population in 1960. So it seems like the people of NB having been eating increasing amounts of moose over the years.
It will be interesting to see if these unlucky folks are moose eaters.
Edit: Link - https://www.cbc.ca/news/canada/new-brunswick/2020-new-brunsw...
The moose population there has increased nearly 10x since 1960. In the 2020 hunting season, 3900 moose were taken. That is more than the entire population in 1960. So it seems like the people of NB having been eating increasing amounts of moose over the years.
It will be interesting to see if these unlucky folks are moose eaters.
Edit: Link - https://www.cbc.ca/news/canada/new-brunswick/2020-new-brunsw...
Wouldn't the doctors have already tested the patients for CWD?
> found that plants can be a vector for prions. When researchers fed hamsters grass that grew on ground where a deer that died with chronic wasting disease (CWD) was buried, the hamsters became ill with CWD
OMG.
I recall watching a PBS show on prions back in the 90s where they tried to destroy them in various ways : burning, burying, etc, and they were extremely difficult to destroy.
OMG.
I recall watching a PBS show on prions back in the 90s where they tried to destroy them in various ways : burning, burying, etc, and they were extremely difficult to destroy.
this is well known. Co-grazing scrapie sheep will cross over into cows as mad cow disease. However, co-grazing CWD deer will not cross over into cows or sheep (this is also well known). The determinants of why X can or can't cross over into Y are not well-understood, yet.
Sodium hydroxide works, but you're basically chemically destroying anything organic. The alternatives include 1,000°C fires and other such fun things.
It's absolutely terrifying not only how dangerous these diseases can be, but also how little research funding they get. If there is ever a widespread prion outbreak, it'll end up making Ebola look like a common cold.
That's actually a terrifying and seemingly feasible dystopian future
Is there any explanation for why this hasn't occurred already?
the world solved the COVID-19 vaccine problem pretty quickly when every country turned their attention to it..... I wonder what modern medicine could do for Prion disease in say.. 2035. I bet we could figure out some cool protective mechanisms.
Incidentally, Kim Stanley Robinson's novel Aurora, which aims to deflate optimism that human beings will ever expand past our solar system, describes an attempt to colonize an Earth-like planet that fails because its environment is rich in prions.
I feel angry after reading your comment. I have that book on my shelf and plan to read it soon. You spoiled part of the story. :(
People discuss book plots online all the time and a person can’t expect to remain oblivious to everything, especially considering that what I mentioned isn’t even a shocking plot twist: it is where KSR goes after this part of the book that will challenge and surprise readers.
And you spoiled an excellent book for others on the site, why?
Mentioning this isn’t a spoiler – it comes fairly early on, and before KSR gets to what are arguably his main points – unless you are one of those people who think that any mention whatsoever of a book’s plot is a spoiler. Plus, it was widely discussed in reviews at the time.
It's in the middle of the book and is a very important spoiler. You very much summarized the first half of the book.
Exactly, "first half of the book" is not spoiler territory. It is the sort of thing typically summarized in a review. One can only reasonably demand a spoiler warning for a twist that comes later in a book. What will surprise readers of Aurora are two things that happen after that prion plot.
Yikes, I grew up in that area (on the U.S. side) and my neighbor down the street passed away from a disease related to mad cow disease about 10 years ago. The article lists mad cow as a variant of Creutzfeldt-Jakob disease...I wonder if this is what he had?
The area is largely agricultural, and the cancer rate for that region is high compared to the rest of the state (Maine). Hmm.
The area is largely agricultural, and the cancer rate for that region is high compared to the rest of the state (Maine). Hmm.
"The article lists mad cow as a variant of Creutzfeldt-Jakob disease"
That's not quite right, though they are both prion diseases. See https://www.cdc.gov/prions/
That's not quite right, though they are both prion diseases. See https://www.cdc.gov/prions/
No mad cow disease is quite literally Creutzfeld-Jakob disease, it's vCJD, with v standing for "variant", to specifically distinguish it from "normal" CJD which is heritable. usually CJD is caused by a specific mutation in the human prion protein that renders its susceptible to turning into plaques; vCJD does not require this mutation and happens in wild type protein, though it's unclear if other heritable factors can contribute to susceptibility.
(I worked in a protein plaque lab and did a small, inconclusive experiment about cross species CJD transfer)
(I worked in a protein plaque lab and did a small, inconclusive experiment about cross species CJD transfer)
Since the article was (I believe) referring to classic CJD, I linked the CDC page because it includes this text:
"Classic CJD is not related to “mad cow” disease. Classic CJD also is distinct from “variant CJD“, another prion disease that is related to BSE."
"Classic CJD is not related to “mad cow” disease. Classic CJD also is distinct from “variant CJD“, another prion disease that is related to BSE."
I don't know why the CDC is saying that, it's just flat out wrong unless the CDC has a really stupid definition of "related".
It's related. They both involve the same protein. If you get a bone marrow transplant from someone with CJD, you will get "vCJD". Presumably also if you eat the brains of someone with CJD. The differential between the two is age of onset.
It's related. They both involve the same protein. If you get a bone marrow transplant from someone with CJD, you will get "vCJD". Presumably also if you eat the brains of someone with CJD. The differential between the two is age of onset.
Good call, thanks for the clarification!
Maybe contact them and report it: https://www.canada.ca/en/public-health/services/surveillance...
Can't hurt.
Can't hurt.
I'll look into it. Thanks for the link!
> The area is largely agricultural, and the cancer rate for that region is high compared to the rest of the state (Maine). Hmm.
That is probably related to Irving (largest landowner in Maine) forestry practices: https://news.ycombinator.com/item?id=26507652
That is probably related to Irving (largest landowner in Maine) forestry practices: https://news.ycombinator.com/item?id=26507652
I grew up there and worked at the NB ministry of agriculture before heading to tech.
While Irving does have a chokehold on everything in NB, there's also a lot of agriculture in NB with very little resources to properly regulate. During my time there, small farmers would spray crops with unregulated herbicides/pesticides/fungicides, and even if we reported them, nothing would get done.
The area was also a huge test site for Agent Orange: https://www.canada.ca/en/department-national-defence/corpora...
People aren't very rich and don't take good care of themselves. The education level is low and most people smoke and the rate of obesity is incredibly high: https://www2.gnb.ca/content/dam/gnb/Departments/h-s/pdf/en/P...
While Irving does have a chokehold on everything in NB, there's also a lot of agriculture in NB with very little resources to properly regulate. During my time there, small farmers would spray crops with unregulated herbicides/pesticides/fungicides, and even if we reported them, nothing would get done.
The area was also a huge test site for Agent Orange: https://www.canada.ca/en/department-national-defence/corpora...
People aren't very rich and don't take good care of themselves. The education level is low and most people smoke and the rate of obesity is incredibly high: https://www2.gnb.ca/content/dam/gnb/Departments/h-s/pdf/en/P...
The area is a agricultural wasteland, scoured by repeated glaciation shoving everything down to and including the granite bedrock into the Atlantic. If nutritional value is coming out of something other than a bog or the ocean then someone first added it in as fertilizer.
The next century of human existence is going to be filled with discoveries about how toxic everyday items are.
Take spray deodorants for example. I absolutely fail to believe that adequate studies were conducted to determine if inhaling small amounts of whatever is in there didn’t cause cancer after 50 years.
Take spray deodorants for example. I absolutely fail to believe that adequate studies were conducted to determine if inhaling small amounts of whatever is in there didn’t cause cancer after 50 years.
I wonder if Chronic Wasting Disease has finally jumped to humans given that NB has a large number of Deer/Moose hunters.
CWD is a prion disease. Mad cow disease is also a prion disease. In humans it’s called vCJD so we know prion diseases in cattle affect people.
All known prion based chronic wasting diseases have been rules out, according to the article.
Not to mention Rappie Pie https://www.thestar.com/life/food_wine/2020/03/17/some-in-qu...
Fuck I hope not. I just googled the CWD occurrence map in the US. It’s...very scary.
[deleted]
"the disease is not genetic and could be contracted from water, food or air."
New Brunswick is basically a lumber colony for the Irving companies (also, so is Northern Maine, see sprainedankels' comment elsewhere in this thread: https://news.ycombinator.com/item?id=26506784). On average 1% of the forest cover of New Brunswick (at least 85% of the province is forest cover) is clear-cut every year, and then the clear cut is drenched in herbicides from the air. The current herbicide is glyphosate, which has been linked to neurological disorders, but aerial spraying of herbicides in other forms dates back to the 1950s.
Here is a linkdump for more information:
http://www.stopsprayingnb.ca/
http://isourforestreallyours.com/Isourforestreallyours/Start...
https://web.archive.org/web/20160322213243/http://dearbriang...
https://www.cbc.ca/news/canada/new-brunswick/forests-and-flo...
https://www.cbc.ca/news/canada/new-brunswick/louis-lapierre-...
https://www.cbc.ca/news/canada/new-brunswick/forestry-deal-r...
This even comes up in the comments section on satire websites:
"Back around 1973 or 1974, I knew of a photographer who had a summer contract with Irving, documenting the effect of spraying. He photographed deformities, poisoned animals, etc. The company was very secretive and made sure that every single roll of film was handed back to them." https://themanatee.net/firing-of-dr-cleary-proves-once-for-a...
https://themanatee.net/new-brunswicks-10-most-beautiful-clea...
New Brunswick is basically a lumber colony for the Irving companies (also, so is Northern Maine, see sprainedankels' comment elsewhere in this thread: https://news.ycombinator.com/item?id=26506784). On average 1% of the forest cover of New Brunswick (at least 85% of the province is forest cover) is clear-cut every year, and then the clear cut is drenched in herbicides from the air. The current herbicide is glyphosate, which has been linked to neurological disorders, but aerial spraying of herbicides in other forms dates back to the 1950s.
Here is a linkdump for more information:
http://www.stopsprayingnb.ca/
http://isourforestreallyours.com/Isourforestreallyours/Start...
https://web.archive.org/web/20160322213243/http://dearbriang...
https://www.cbc.ca/news/canada/new-brunswick/forests-and-flo...
https://www.cbc.ca/news/canada/new-brunswick/louis-lapierre-...
https://www.cbc.ca/news/canada/new-brunswick/forestry-deal-r...
This even comes up in the comments section on satire websites:
"Back around 1973 or 1974, I knew of a photographer who had a summer contract with Irving, documenting the effect of spraying. He photographed deformities, poisoned animals, etc. The company was very secretive and made sure that every single roll of film was handed back to them." https://themanatee.net/firing-of-dr-cleary-proves-once-for-a...
https://themanatee.net/new-brunswicks-10-most-beautiful-clea...
The people responsible for the damage causes by the herbicides will be long gone and their decedent's wealth safe before anyone will ever get held responsible.
Chevron (Texaco) killed and poisoned hundreds and when they finally lost in court they went after the laywer which is currently still under illegal house arrest in NY. [1]
[1] https://www.youtube.com/watch?v=a5RZLyLCTo0
Chevron (Texaco) killed and poisoned hundreds and when they finally lost in court they went after the laywer which is currently still under illegal house arrest in NY. [1]
[1] https://www.youtube.com/watch?v=a5RZLyLCTo0
Talk about jumping to conclusions.
As a biochemist (no conflict of interest), I honestly don't think there's any reason to think this has anything to do with glyphosate or herbicides.
Environmental exposure in general is unlikely to have a subacute presentation such as in CJD, so that probably rules out exposure to reasonable levels of any herbicide.
As a biochemist (no conflict of interest), I honestly don't think there's any reason to think this has anything to do with glyphosate or herbicides.
Environmental exposure in general is unlikely to have a subacute presentation such as in CJD, so that probably rules out exposure to reasonable levels of any herbicide.
Thank you for your expert input. I agree - this is just uninformed speculation on my part. I thought I should point out the unusual circumstances in New Brunswick.
Has glyphosate or pesticides ever been linked to prions?
As far as I can tell only very tangentially. There is this study: https://people.csail.mit.edu/seneff/2017/SamselSeneff_Glypho...
And possibly exposure to chronic wasting disease from deer being driven from forests (Irving re-plants clear-cuts with spruce monoculture that provides no food for deer) into proximity to humans:
https://www.cbc.ca/news/canada/new-brunswick/deer-st-andrews...
https://www.cbc.ca/news/canada/new-brunswick/deer-mobs-bathu...
https://www.ctvnews.ca/canada/n-b-town-overrun-by-deer-consi...
And possibly exposure to chronic wasting disease from deer being driven from forests (Irving re-plants clear-cuts with spruce monoculture that provides no food for deer) into proximity to humans:
https://www.cbc.ca/news/canada/new-brunswick/deer-st-andrews...
https://www.cbc.ca/news/canada/new-brunswick/deer-mobs-bathu...
https://www.ctvnews.ca/canada/n-b-town-overrun-by-deer-consi...
Seneff has dubious credibility on this subject.
Interesting. Will take a look at the links, thanks for the info!
I'm not sure why you're linking all these when the disease has been identified to most likely be a prion disease, and not a side-effect of herbicides.
In terms of chronic exposure of the majority of the population to herbicides, New Brunswick is fairly unique. Why do you think that it is not a plausible hypothesis for "an unknown neurological disease"? If this is a new prion disease from game animals, why has it not shown up in Maine, Québec, or Vermont?
How is it unique?
I used to work for the Ministry of agriculture there and we shared most of our data and procedures with Quebec's.
I used to work for the Ministry of agriculture there and we shared most of our data and procedures with Quebec's.
Are you serious?
Québec land area: 1,365,128 km²
Québec total annual timber harvest: 4.8 million m³
New Brunswick land area: 71,450 km²
New Brunswick timber harvest from Crown lands: 5.4 million m³
New Brunswick timber harvest from private lands: 3 million m³
Québec protected areas alone are more than twice the size of the whole of New Brunswick.
What other populated region in North America is as intensely logged as New Brunswick? Maybe rural Oregon or Washington?
Québec land area: 1,365,128 km²
Québec total annual timber harvest: 4.8 million m³
New Brunswick land area: 71,450 km²
New Brunswick timber harvest from Crown lands: 5.4 million m³
New Brunswick timber harvest from private lands: 3 million m³
Québec protected areas alone are more than twice the size of the whole of New Brunswick.
What other populated region in North America is as intensely logged as New Brunswick? Maybe rural Oregon or Washington?
Yeah, but rural PNW is mountainous valleys. Lots of geographic separation from population centers in terms of tree farming areas. If it’s some sort of “runoff” from logging, unless it hits the water table (water tends to come from wells, not lakes/rivers) it’s unlikely to be easily exposed in the populace.
The article we are talking about says specifically that no prion diseases tested for have been found and that readers should not jump to the conclusion that this is a prion disease.
[deleted]
I was hoping not to see CJD or prions mentioned in this article.
I wonder if these tests for prion diseases included chronic wasting disease that occurs in deer populations. Is there a lot of deer hunting in New Brunswick? If this is a prion disease and it is caused by the same prion as chronic wasting disease, a lot of people are going to die in north america.
I wonder if these tests for prion diseases included chronic wasting disease that occurs in deer populations. Is there a lot of deer hunting in New Brunswick? If this is a prion disease and it is caused by the same prion as chronic wasting disease, a lot of people are going to die in north america.
>Creutzfeldt-Jakob disease and some of its variants, including mad cow disease, or bovine spongiform encephalopathy, or BSE.
I thought CJD and BSE were the same thing.
I thought CJD and BSE were the same thing.
vCJD is the human variant of BSE.
Not being a biologist, I'll ask what might be a dumb question: If the essential elements of each disease are compared, is there a difference? Are the prions associated with each disease different from one another? If so, is there a difference elsewhere that defines between one disease and the other?
Here's good information on the matter: https://emedicine.medscape.com/article/1169688-overview#:~:t....
Thanks! I did a bit of digging around, and it looks like the BSE-causing prion can rarely, on ingestion, cause prion formation that causes CJD. So, if I understand correctly, it's not exactly a disease that transmits like a virus, it's one that is induced by the transmission of a diseased particle (the cow prion) when it goes form cows to humans. BSE and CJD seem to be the result of the transmission and induction of the respective prions. It seems comparable to a cow being on fire, and a human catching fire from it -- the latter is a human fire, and the former is a cow fire. Unless I really got that wrong, interesting!
Not entirely related, but how many new diseases are discovered every year ? For example we have new coronavirus every now and then, but what else ?
I don't know the answer, but I suspect this is actually multiple questions:
- How many diseases do we learn to distinguish (i.e. from other diseases) every year as our biological laboratory technology improves. You can imagine that once upon a time, there were multiple strains of coronaviruses in humans, but we had no way to tell them apart (the strains look the same to whatever scale of study is available), which we can now tell apart.
- How many diseases do we discover as we apply modern medical technology to an ever-wider portion of the global human population (again, that are already in humans but which are "discovered" in that year)?
- How many new diseases develop for humans every year? For example, we have no evidence that any of the current strains of Covid-19 were present in humans prior to late 2019.
- How many diseases do we learn to distinguish (i.e. from other diseases) every year as our biological laboratory technology improves. You can imagine that once upon a time, there were multiple strains of coronaviruses in humans, but we had no way to tell them apart (the strains look the same to whatever scale of study is available), which we can now tell apart.
- How many diseases do we discover as we apply modern medical technology to an ever-wider portion of the global human population (again, that are already in humans but which are "discovered" in that year)?
- How many new diseases develop for humans every year? For example, we have no evidence that any of the current strains of Covid-19 were present in humans prior to late 2019.
Define "Discovered". Transmitting in humans? Or transmitting in wildlife? Because for the second, the answer is a tremendous number.
It's mad cow and I don't believe that the US has had only 6 cases of mad cow with the insane way cattle get treated.
They just call them "downers" and terminate them and some most likely end up in the food supply.
They just call them "downers" and terminate them and some most likely end up in the food supply.
You may be right about the US Mad Cow disease outbreak, but they specifically eliminated that possibility here (middle of the article):
The head of a research group on the subject, neurologist Alier Marrero of Moncton's Dr. Georges-L.-Dumont University Hospital Centre, said in an interview: These are patients who have clinical features that correspond to prion diseases, of which Creutzfeldt-Jakob disease is one, but show no evidence of having Creutzfeldt-Jakob disease or any other form of prion disease.
The head of a research group on the subject, neurologist Alier Marrero of Moncton's Dr. Georges-L.-Dumont University Hospital Centre, said in an interview: These are patients who have clinical features that correspond to prion diseases, of which Creutzfeldt-Jakob disease is one, but show no evidence of having Creutzfeldt-Jakob disease or any other form of prion disease.
It says in the article that it's not CJD, but it's possibly a new prion-based disease.
The earliest articles about HIV/AIDS[1] and COVID-19[2] both had similar tones to them ("unknown", "similar to", "possibly a new disease", etc.)
[1] https://www.nytimes.com/1981/07/03/us/rare-cancer-seen-in-41...
[2] https://www.nytimes.com/2020/01/06/world/asia/china-SARS-pne...