[HN Gopher] New Brunswick monitoring more than 40 cases of unkno...
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New Brunswick monitoring more than 40 cases of unknown neurological
disease
Author : curmudgeon22
Score : 100 points
Date : 2021-03-18 14:51 UTC (8 hours ago)
(HTM) web link (www.cbc.ca)
(TXT) w3m dump (www.cbc.ca)
| sprainedankles wrote:
| 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.
| ars wrote:
| Maybe contact them and report it:
| https://www.canada.ca/en/public-health/services/surveillance...
|
| Can't hurt.
| sprainedankles wrote:
| I'll look into it. Thanks for the link!
| tyingq wrote:
| _" 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/
| sprainedankles wrote:
| Good call, thanks for the clarification!
| dnautics wrote:
| 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)
| sedachv wrote:
| > 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
| m00x wrote:
| 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...
| goatcode wrote:
| >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.
| ThePowerOfFuet wrote:
| vCJD is the human variant of BSE.
| goatcode wrote:
| 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?
| m00x wrote:
| Here's good information on the matter: https://emedicine.me
| dscape.com/article/1169688-overview#:~:t....
| wiz21c wrote:
| 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 ?
| InitialLastName wrote:
| 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.
| en4bz wrote:
| I wonder if Chronic Wasting Disease has finally jumped to humans
| given that NB has a large number of Deer/Moose hunters.
| stonecraftwolf wrote:
| Fuck I hope not. I just googled the CWD occurrence map in the
| US. It's...very scary.
| mc32 wrote:
| 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.
| bryanlarsen wrote:
| All known prion based chronic wasting diseases have been rules
| out, according to the article.
| frabbit wrote:
| Not to mention Rappie Pie
| https://www.thestar.com/life/food_wine/2020/03/17/some-in-qu...
| goda90 wrote:
| >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.
| thatguy0900 wrote:
| 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
| bawolff wrote:
| 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)
| thatguy0900 wrote:
| Didn't think about that, fair point
| JauntTrooper wrote:
| 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.
| droopyEyelids wrote:
| Also, the prions themselves are not easily deactivated, and
| can linger in the environment, in an infectious state, for
| decades.
| me_me_me wrote:
| 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
| dwohnitmok wrote:
| 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.
| michael1999 wrote:
| 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.
| sedachv wrote:
| "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...
| sprainedankles wrote:
| Interesting. Will take a look at the links, thanks for the
| info!
| sschueller wrote:
| 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
| m00x wrote:
| 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.
| graeme wrote:
| Has glyphosate or pesticides ever been linked to prions?
| sedachv wrote:
| 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...
| tyingq wrote:
| 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
| ahelwer wrote:
| 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.
| Skgqie1 wrote:
| That's actually a terrifying and seemingly feasible dystopian
| future
| UncleOxidant wrote:
| > 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.
| dnautics wrote:
| 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.
| Mediterraneo10 wrote:
| 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.
| abraxas wrote:
| And you spoiled an excellent book for others on the site,
| why?
| Mediterraneo10 wrote:
| 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.
| abraxas wrote:
| It's in the middle of the book and is a very important
| spoiler.
| davesque wrote:
| 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.
| [deleted]
| ben_w wrote:
| 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?
| dnautics wrote:
| 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)
| greenwich26 wrote:
| 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?
| dnautics wrote:
| 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.
| davesque wrote:
| No idea, honestly. As I mentioned, I'm not an expert.
| subungual wrote:
| 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.
| dwohnitmok wrote:
| > 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.
| JauntTrooper wrote:
| Here's an article on it:
|
| https://www.scientificamerican.com/article/surgical-
| exposure...
| theideaofcoffee wrote:
| 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.
| jhayward wrote:
| They're hard to 'kill' because they were never alive. They're
| just a protein molecule.
| tyingq wrote:
| Ok, "render harmless". FWIW, "kill off" has pretty broad
| established usage for non living things :)
| sschueller wrote:
| 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.
| toss1 wrote:
| 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.
| m00x wrote:
| It says in the article that it's not CJD, but it's possibly a
| new prion-based disease.
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