Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims
blogs.scientificamerican.com
blogs.scientificamerican.com
Iatrogenic transmission is a rarer subset still: the riskiest transplants (with respect to CJD) are in the central nervous system. The UK tracked their transmission rates from 1970 to 2003 (a long period is needed because surgical transmission usually occurs in folks with long incubation periods - up to ten years or so) for dura transplants (something that goes into the CNS) and found a total of eight.
This article is one good step above scare-mongering, but only one.
(1) there are only so many resources to go around. Should we be devoting them to vanishingly rare things rather than things that might actually affect you?
(2) prions will never be a wildfire plague. They have little or no tranmissive ability, by nature of being absolutely naked protein. One step up from that - encoding the protein for space constraints and encapsulating it - is called a virus.
> In a particularly vexing twist, prions are also nearly impervious to destruction, even when attacked using a strenuous combination of disinfectants, heat, and pressure.
Not really. The point your parent is making is that prions themselves don't reproduce like viruses and bacteria. Its basically impossible for a large scale breakout to occur and spread.
They can't reproduce outside of the body, but neither can viruses.
It’s orders of magnitude less reproduction.
From my (ignorant, despite being interested in prions for decades now) perspective, you have something that (1) can replicate, (2) is durable (3) is harmful, and only the relative rarity of prions in general (because 1 and 3 are physically unusual to combine) have kept them from being a general problem. As we tinker with DNA and thus look into creating new proteins and factors that would influence protein-folding, I'd think it's worth taking the matter seriously (though not fear-mongering), certainly as part of our need to understand proteins better anyway.
What are my faulty assumptions?
A virus can overtake a cell and mobilize its resources to create a bajillion copies of that virus. A prion can transform only as much copies of that protein as that cell has made in its normal processes.
A virus requires a cell to replicate, bacteria require a much more limited range of hospitable environments.
Both bacteria and viruses "attempt" to create exact copies, just as a prion does. Admittedly prion replication probably has more accuracy, but that prions occur at all indicates the process allows for natural selection.
These don't seem to put prions outside of the "replication" category - they replicate by encountering other proteins, so they are in a body. Their rate of replication is limited (or boosted) by the rate of encountering these proteins. They attempt to create copies of themselves with the chance of failure or different end result.
>"combining autoclaving (even at 121 degrees C) with a sodium hydroxide treatment is extremely effective." https://www.ncbi.nlm.nih.gov/pubmed/10658760
Usually there is some sodium hydroxide in standard sodium hypochlorite bleach solution as well though, perhaps that explains why they reported it is the most effective of the chlorine bleaches.
One of the characteristic features of prions is their resistance to a number of normal decontaminating procedures. These pathogens are resistant to processes affecting nucleic acids, such as hydrolysis or shearing [9]. However, agents that digest, denature, or modify proteins do have activity against prions [7]. The PrP purified from the brains of scrapie-infected animals (PrPSc) can be inactivated by prolonged autoclaving (at 121ºC and 15 psi for 4.5 hours), or immersion in 1N NaOH (for 30 minutes, repeat three times), or immersion in concentrated (>3 M) solutions of guanidine thiocyanate [10]. However, certain cautions prevail; it appears that inadequate autoclaving can establish heat-resistant subpopulations that fail to diminish with a further cycle of autoclaving [11]. Stainless steel instruments also may retain infectivity even after treatment with 10 percent formaldehyde [12,13].
Newer decontamination techniques are being investigated. There has been some success in sterilization using a combination of sodium dodecyl sulfate (SDS), proteinase K, and pronase [14]. A radio-frequency gas-plasma treatment has been shown to effectively decontaminate surgical instruments [15]. Another group has tested a decontamination formula combining copper metal ions with hydrogen peroxide [16].
As it is, very few proteins in humans have alternate favorable form (by luck or selection?). The exception appears to be the extremely complex and relatively new nervous system of mammals. I'd guess that our immune systems might be another place to look, but that is very highly tested.
Frankly, this seems like a susceptibility that is rare because it is chemically odd not that there is a lack of potential exploitation. Luckily, the range of creatures that share our respiration system far exceeds the range of creatures which share our recently evolved nervous system.
https://www.theguardian.com/society/2018/dec/10/mysterious-p...
Chronic wasting disease (CWD) (affects deer) can be transmitted by infected saliva. One of the symptoms of symptoms of CWD is excessive saliva production. It is very transmissible, and is devastating captive populations of deer, although it is relatively rare in the wild.
I think if a similar TSE affected humans, spreadable by infected saliva, it would absolutely be a wildfire plague.
At first I assumed that 'prions' came in many flavors, like virus. However from my brief lit search, it seems like people are talking about (p)rion proteins as if they were't all (P)rion proteins (PRNP). If all mammalian 'prion' disease can be traced to a single conserved gene, I think the risk of another 'prion-like' disease cropping up is pretty low.
But then again, who knows? Prion disease still seems shrouded mystery, which I find odd given (1) how inherently interesting this topic seems, and (2) we've gotten really, really good at molecular biology.
I feel like we are missing a key piece of the puzzle when it comes to understanding prion disease. The literature seems to want people to conceptualize the disease etiology as such: an alternately folded (disease causing) variant of PRNP protein (PrPs) bumps into a normal version of PRNP (PrPc), causing a normal copy of PrPc to also misfold into PrPs. Rinse, repeat. So you can basically think of it like an enzyme that catalyzes a conformational change on versions of itself, in a feed-forward cascade. I feel like an alternative hypothesis however, could be one of an autoimmune response. The mammalian immune system, particularly the antibody system, is mind blowing. This system can recognize virtually any foreign protein -- it can even recognize specific sub-moieties and conformations of proteins (antigens). It must, do this while never mistaking an endogenous protein to be foreign; if it does, you get an autoimmune disease. My idea is basically that PrPs is just slightly different enough from PrPc to get tagged as foreign by the antibody system (which it absolutely can do, given that we can perform western blots to detect the PrPs variant), but also just similar enough for the antibody system to mistake some endogenous PRNP for the foreign version.
What if this were suddenly transmissible to humans?
https://en.wikipedia.org/wiki/Scrapie
"In the United Kingdom, the government has put in place a National Scrapie Plan, which encourages breeding from sheep that are genetically more resistant to scrapie. This is intended to eventually reduce the incidence of the disease in the UK sheep population."
Mad Cow Disease was caused by a prion[0].
[0]:https://en.wikipedia.org/wiki/Bovine_spongiform_encephalopat...
The phrase is actually “deep seated.”
Merriam-Webster at least (the only one I've checked) says that "deep-seated" is the only correct version:
Definition of deep-seated
1 : situated far below the surface
2 : firmly established
https://www.merriam-webster.com/dictionary/deep-seated
“deep-seeded”
The word you've entered isn't in the dictionary.
https://www.merriam-webster.com/dictionary/deep-seededP.S. Inconsistency: I assumed that whiddershins was complaining about the hyphen, rather than correcting ‘seeded’, which had been changed to ‘seated’ by the time I first saw it.
I'm not sure there will ever be a prion that hasn't already existed, whereas I am sure there will be new viruses and bacteria.
A prion only makes itself. Even if it replicated imperfectly, the odds of a slightly different misfolded protein becoming another prion are vanishingly small (otherwise we'd all be dead) because there probably just are very, very few configurations possible. The vast majority of misfolded proteins just do nothing.
There are several billion humans who are each replicating millions of copies of each of the 20,000 or so proteins during their lifetimes. It seems pretty unlikely that there are many misfolds that haven't occurred. It's just that only a handful become prions. And even if they do, prions are thankfully very hard to communicate (again, we'd all be dead if they weren't).
Well now I have new nightmares... :)
But in seriousness, I don't know much, but I believe that protein-folding is a hugely complicated problem that still lies at the edge of our understanding, therefore I conclude that we can't really conclude that the options for a replicating, harmful prion are basically harmless.
I mean...we don't even really know how common prions are, right? We only have noticed a handful because they have dramatically bad symptoms. A large number of replicating but harmless prions could exist (right?).
Obviously there's no reason to panic, but considered caution and study seems a better reaction than overconfident dismissal.
Whether that replication can be incorrect and produce a different kind of prion, I don't know. If it can, evolution will do its work.
Given that each human replicates each protein many millions of times over the course of their life, and that there have been several billion humans alive, if that were possible we'd probably have experienced it by now and again all be dead.
> In a particularly vexing twist, prions are also nearly impervious to destruction, even when attacked using a strenuous combination of disinfectants, heat, and pressure.
Regardless, it's unrelated to prions.
https://www.newscientist.com/article/2151909-alzheimers-may-...
So while wiring together two bloodstreams is a bit extreme, and likely to result in most things being transmissible, there's nonzero evidence that you can have a bad day from a blood transfusion.
I agree. Could a moderator please change the title at the least?
"Prions, A Rare and Lethal Infection, ..." would be slightly better.
http://www.virology.ws/2015/06/12/resistance-to-prion-diseas...
Many on this board point to the inelasticity of their transmissible potential and call this fear-mongering. However, it is that same inelasticity that makes them intransigent. Once they have compromised an instrument, that instrument will remain compromised until each prion has specifically been destroyed. When you pair that intransigence with its lengthy incubation period, you actually have a force multiplier that, while not viral, would be scary enough to change behavior.
Fortunately, the article hints that retinal scans may eventually be used for initial diagnosis.
Isn't that begging the question? I mean, the author's concern seems to be that the prevailing belief hasn't been well-tested compared to the potential impact of it being wrong.
It's already happened before: There was a period of time when most medical practitioners were adamant that their actions could possibly be transferring disease, and it was an uphill fight to get regular disinfectants and hand-washing.
* Kuru (laughing disease) was transmitted from cannibalism. Women and children of the tribe were more likely to contract it because they were more often given the less palatable parts of the bodies, which included brains
* Some countries do not trust American cattle because our standards for Prion screening are too low
* Kentucky has issues with Prions because Squirrel brains are a popular dish
Try not to eat animal or human brains and you can dramatically reduce your chances of getting Prions
Or is that the only organ where they manifest in symptoms?
Looks like the dish is called Burgoo
> The center of the burgoo universe is Owensboro, Kentucky
Thanks!
I am going to arrange a family event where that happens.
Those are,after all, the same things we (rightfully) laud in the consumption of other types of more normative food animals. Just because it's a squirrel shouldn't change that.
EDIT: Added editorial wording to second paragraph.
Maybe it is an issue but goes underreported? Prions related diseases were historically easy to misdiagnose because they can present in so many different ways. I'm sure medical science has caught up in 1st world countries but maybe lags elsewhere?
Now all I can think about is the dinner scene at the end of Hannibal.
Is it a big issue out there?
If I've eaten sheeps brain twice myself, how soon should I plan my funeral?
iirc, incubation periods can be decades long. I believe the more you injest, the shorter the incubation period
Honestly there’s a lot of fear mongering in this thread about brain consumption...if anything it’s healthy for you. The main issues are most likely when the animal eaten has participated in cannibalism. For example, Cows were fed bovine blood meal which is what led to the Mad Cow scare, kuru is caused by cannabilistic human tribes, squirrels are known to be cannibalistic, etc. Lamb/sheep don’t participate in cannibalism. No need for unnecessary fearmongering
Scrapie is a fatal, degenerative disease affecting the central nervous system of sheep and goats. It is among a number of diseases classified as transmissible spongiform encephalopathies (TSE). Infected flocks can experience significant production losses.
* Do we have a good grasp on the number of prions in the world? My recollection from when MadCow became a big thing in the late 90s was that prions weren't even looked for and were hard to find as a general rule, but that was 20+ years ago. Might there be many common-but-harmless prions that we haven't even noticed?
* WHY is brain consumption (both in humans and in animals) such a source of prions? What makes that situation more likely to create prions?
From what I remember on the topic, we do not have a good grasp on prions disease. There is a good chance that some other diseases in history may have actually been misdiagnosed prions disease. It can present as a form of dementia and the incubation period can be so long that many people are middle-aged before symptoms occur.
Tissue of the brain and spine are better for Prions propagation. Prions cause other proteins to misfold themselves. The proteins they affect are related to brain matter.
People talk about Mad Cow, Kuru, Creutzfeldt-Jacobs, Chronic Wasting Disease, Scrapie, and a dozen other diseases like they are not all manifestations of the same underlying problem with PRNP gene variants.
1. Symptoms show up soonest in brains and so can be distinguished from other causes of impairment or death.
2. Humans already some immune mechanism that helps protect against prions, but brain tissue is particularly susceptible because of it's less-usual immune-system setup.
http://www.cureffi.org/ and http://www.prionalliance.org/blog/
> Wife-husband team Sonia Vallabh and Eric Minikel are on a very personal mission to find a cure to prion diseases. When Sonia was diagnosed as a genetic carrier of a rare disease called Fatal Familial Insomnia (FFI), she and Eric quit their regular jobs and began studying molecular biology to learn as much as they could.
Theirs is an incredible story so far. https://www.newyorker.com/books/page-turner/a-prion-love-sto...
There are a few comments here about how talking about prion diseases are basically fearmongering. It shook me a bit, though. I always thought of CJD as one of those freak diseases that you hear about in the news, but is such low likely hood that it's basically non-existent. Not so much anymore.
Are you sure it's CJD? Article says 95% of CJD patients won't live 12 months after diagnosis.
> Another family member that was a nurse in Kentucky(?) said they have a high rate of prion disease due to farmers inhaling contaminated dirt for years.
What kind of prion disease and what kind of dirt?
The nurse family member said cow farmers, so I assume cow feces contaminated dirt, and that their brains looked "like Swiss cheese". This was also in the context of mad cow disease. I checked my notes from the day and it was North Carolina, not Kentucky.
If you eat brains, your risks go up by multiple magnitudes.
More likely is that my eye problems are from wearing contacts in for too long and staring too long at a computer in a low-humidity environment and never blinking. My cognitive issues are probably from not eating healthily, not getting enough sleep, not exercising, and from being depressed.
You could also explain this with low-level heavy metal exposure, e.g. mercury, as well as a large amount of other possibilities :-)
I thought hard if I should write this, as a joke and only the above line at first, but here it goes. Having read your second paragraph too, that is pretty much exactly what I said to myself a decade ago and before. Then some things escalated a little bit and I was forced to either accept all my increasing issues, or do some research myself.
Turned out I had chronic heavy metal poisoning from mercury. Fortunately diagnosis was made easy because I actually had high levels of it in hair and blood (and urine - same as blood since that's where that comes from). Anyway, the scale of the problem was only made clear during chelation therapy, when both I as well as my (university clinic researcher) doctor were amazed at all the things that improved, including "miracles" such as thyroid normalization (had been enlarged and with a nodule for decades, completely gone within half a year of DMPS chelation, left the endocrinologist speechless, literally, he checked me twice with ultrasound to make sure he had not make a mistake). This issue is next to impossible to diagnose with today's methods, unlike acute poisoning, because how much you see in blood, urine or even hair does not say anything about the size of the problem, it only shows up when it actually moves around the body which it only does after exposure.
I had had dry eyes too, and of course the ophthalmologist as well as I myself attributed it to too much screen work. Today, after years of chelation, I sit in front of a monitor much more than ever before - but now I have zero issues. Same with RSI. Sooo many little things are completely gone from my body, depression too.
The crazy thing is, up to that point ~10 years ago when I had to confront that something was not quite right, I called myself "healthy". And why not? I could run a half marathon just for fun and still come back feeling relaxed. All the many little issues I had, such as a few warts on the feet (more and more over the decades but I ignored the trend), winter depression (many people have that, it's normal!), eye problems (not really severe, so not worth thinking about it) and many many other, my brain refused to think about it. The reality my brain created of my world was highly distorted, but I did not know it. Only the huge and unexpected changes happening after I began chelation (DMPS, later DMSA) showed me the extend of the thus far ignored problem.
The first thing I found was, after having first dismissed it as esoterics when I found it on the net, that I had a candida problem. Proven by the significant immediate effect of taking Nystatine (anti-fungal) and a doctor confirmed it. Later I even got Fluconazole, an internal antifungal, again with huge effects. Just an aside, funnily enough, the warts on the sole of my feet that every doctor would say were caused by a virus disappeared by 95% after Fluconazole.
Anyway, I learned Candida never is a cause but itself just a symptom, and since I had none of the conditions that are listed in the text books that might cause it I had to continue looking. Heavy metal poisoning was one of the only options that would explain the Candida, but also everything else. And I had only one possible source: amalgam fillings.
When the tests confirmed high levels of mercury in my hair as well as in my urine I still was not completely sure: It was high but not high enough to explain the severity of my issues. The doctor I went to said it was high enough to justify starting chelation, but I must have something else. I had the remaining three fillings removed with special equipment and protection. I had had three or four removed decades earlier without protection, just normal drilling, at the beginning of my CS study, and now something made sense: At the time I had suddenly, out of nowhere and pretty much overnight, developed almost-asthma level pollen allergy, and for half a year I had severe problems with the right leg joint. I never went to a doctor for the joint because the symptoms clearly showed me it was not "physical", I can't explain it with a few words. I also developed severe insomnia and - as I understand now but not at the time - "dumb". I had always been good at math, for example, but suddenly it was a problem. Stuff that today, after all my chelation, again looks very easy to me. I still passed and I was still very good at everything more practical so getting through the study was no problem. At the time I completely ignored all those sudden issues and took it as "that's just live".
Everything started to improve greatly during the first year of chelation, and after the fifth or so DMPS treatment in the following weeks the tissue around my right-side thyroid became very active. That was very curious, because I had had a double-sized right thyroid with a nodule for at least two decades, re-diagnosed just a year prior. Recommendation back then was surgery to get rid of the nodule. The tissues in that area became active after two or three DMPS treatments. I went to the endocrinologist again when I felt nothing else there. He did an ultrasound, and when he was done he started casually looking through his notes and became more and more agitated. He asked me to lie down again and he repeated the ultrasound, right side only this time. At the end the result still was this: Thyroid size nearly completely normal, and the nodule had disappeared completely. That was the point when I actually believed that yes, I had found the source of the problem.
Regarding an even more clear proof for amalgam fillings as the cause:
After about a year the doctor wanted to make an injection in to my buccal mucosa. It's just mucosa, so you don't need any force, but his needle ended up deep inside jaw bone. He found several places where the needle easily penetrated into bone. All those places where where amalgam fillings had been for two or three decades. He injected DMPS and just like in the thyroid area the are began to "work". Very actively. A year later the needle could not penetrate anywhere any more. The jaw remained a very very active area though for years, and it still reacted to chelation. An aside: The are had been OPG x-rayed a few times in the years before because I had finally gotten my crooked teeth fixed and jaw extension surgery too (now I have perfect bite and teeth, wonderful!!!). No doctor ever saw anything in my jaw. One wasn't sure about seeing a tiny shadow near one root - the one where I had the most issues and that was the most active later during chelation - but didn't take it serious enough and didn't follow up (when patients don't complain why would you if it's so subtle). But bone that can be penetrated by a needle easily is very broken. This shows how much (more) broken something has to be to show up even in an OPG x-ray.
My doctor never found anything else and now he too thinks that the only problem there ever was were the amalgam fillings. A lot - a lot! - of very interesting stuff happened to me, the chelators only paved the way out and started it, 99% of the actual work was done by my body (most chelators only work in extracellular space anyway). For example, there were three months of nightly 3-4 am kidney pain, but after the nephrologist found that despite elevated protein levels in urine there was no sign of kidney disease I didn't worry about it. It fit into the pattern of stuff happening at various times all over my body and my brain, a new phase starting every few months in another area of the body. For those three kidney pain months I simply got up when it started at 3 am or so, went to bed when it stopped at 5 am and slept until 10 and still got my sleep. No problem.
Even tough I'm a CS person, I actually have a background not in medicine, but (for fun and out of interest) I've also have many courses in biology, chemistry, org.chemistry, biochemistry, anatomy, physiology, neuroscience, several courses in each. I don't care about pathologies, I only wanted to understand the basics of biology and life and of human physiology.
Ageing? And basically what you describe. I wouldn't worry about it :)
In 2017, there was a Canadian study that seemed to show that feeding macaque monkeys raw muscle meat from infected deer was capable of transmitting the disease. I don't think the study has been published yet, but it's described in detail in this Norwegian report (https://vkm.no/download/18.25950e0715e84a118a6ec492/15069251...) and forms the basis of an advisory by the Canadian government (https://www.thetyee.ca/Documents/2017/06/24/Risk-Advisory-Op...) suggesting that consumption of all potentially infected meat be avoided.
Then in 2018, a separate seemingly very similar American study was published (http://sci-hub.tw/10.1128/JVI.00550-18) , saying that no evidence was found for transmission to macaques, even after direct cerebral exposure. The NIH issued a press release (https://www.nih.gov/news-events/news-releases/nih-study-find...) with the headline "NIH study finds no chronic wasting disease transmissibility in macaques".
Suffice it to say, there seem to be contradictory messages here. Is there some overview that would help to clear things up?
> To date, in the US, there have been no known cases of variant CJD, or human prion disease acquired by eating contaminated beef. Here at the NPDPSC, we have diagnosed 4 cases, but they were all acquired out side the US; 1 case in 2044 acquired in UK, 2 cases in 2006 acquired in Saudi Arabia, and 1 case in 2014 acquired in the middle east.
> In several western US states, Canada, and now South Korea and Norway, there is concern that chronic wasting disease (CWD), the prion disease of elk and deer, may be transmissible to people who hunt, butcher, or eat the affected animals. Although transmission of CWD from animals to humans is unlikely, recent data indicate that the barriers between species may be weakened when CWD has been transmitted from animal to animal several times (as may happen in the wild). The NPDPSC, along with the CDC, Department of Health, and the Game and Wildlife Administration, is carefully monitoring any possible case of acquired CWD in humans.
Asked for extra clarification, "So although there is a concern with CWD crossing that barrier, there still haven't been any recorded cases?"
> That is correct.
[1] https://www.the-scientist.com/features/the-bright-side-of-pr...
There were also human cases getting reported at a rate that some suggested matched the pattern for the early days of an epidemic.
Luckily that never materialised. Still though having lived in the UK during a certain time period precludes one from donating blood elsewhere, to this day.
An epidemic is by definition a statistically significant deviation from historic baselines.
Every single upswing within the normal rates of variability - all of the sin curve living above 0 - looks like “early epidemic.” We have one every month.
That was scare-mongering, too. Everyone loves to report an incipient disaster, at least until people get numb and the ratings fall down.
> patients with very long incubation periods of at least 34 years, and probably more than 40 years.
https://www.thelancet.com/journals/lancet/article/PIIS0140-6...
https://en.wikipedia.org/wiki/Cattle_feeding#Mad_Cow_Disease
Compare US position: https://www.feednavigator.com/Article/2017/07/22/USDA-Feed-n...
VIC position: http://agriculture.vic.gov.au/agriculture/livestock/beef/fee...
"Undiagnosed CJD patients may seek testing. And the diagnostic equipment used to test them may then become contaminated, the authors write. They recommend single-use instruments or the adoption of new decontamination procedures for opthalmological equipment with better effectiveness on prions."
In my last several visits, nothing has touched my eye at all.
I don't completely understand your answer, due to my ignorance of the immune system. I thought the immune system could target specific proteins (e.g. how snake venom vaccines work) rather than using the proteins to identify target cells. Is this correct? I as assuming such a mechanism would be able to do something useful with the target.
The other part of your answer I did understand, and that was helpful, thanks!
Or are the abnormal prions in CJD and the like not uniform enough for general prophylaxis?
I really have a naive understanding of immunotherapy, so sorry if my question is dumb.
I did some more cursory research. Interestingly I was half-wrong about the immune system - while I was right about it not being equipped to stop it can do something. Monoclonal antibody treatments delay it. However another problem is that the body's own proteins which are deformed into prions aren't recognized as foreign bodies. Given that notes mention that mice vaccines only delay the onset along with monoclonal antibody treatments. Some sources seem to indicate that it only blocks prions from deforming as opposed to eliminating combined with the fact that native proteins will keep on cascading.
I wonder how much processing power would be required just to get a candidate 'prion grabber' - never mind going through ones which won't turn out to be horrifyingly toxic in other ways.
Here are a few sources I skimmed I would be glad to know where I went wrong.
https://www.researchgate.net/publication/233941323_Immunothe...
FWIW, both my parents had cataracts removed, and my father who'd worn eyeglasses since the 1940's said he'd never seen better in his life. So, chin up.
> we estimate that the mean duration of the incubation period is 16.7 years
https://www.thelancet.com/journals/lancet/article/PIIS0140-6...
> A comprehensive statistical analysis had earlier reported that the mean incubation period was between 10.3 and 13.2 years, but the tail of the distribution was long. Using these estimates, we calculated that the upper 0.4% quantile was 40 years, and even larger for those infected young (>50 years), very much in line with these new observations.
> them may then become contaminated, the authors write. They recommend single-use instruments or the adoption of new decontamination procedures for opthalmological equipment with better effectiveness on prions.
https://www.allaboutvision.com/conditions/cataract-surgery.h...
Are there statistics on Creuzfeld-Jakobs in Langley?
I understand how something like bacteria or a virus enters a host and replicates, but an anomalous protein corrupting other protein is just so strange. I'm sure science will adequately explain it someday but until then it's a pretty WTF anomaly to me.
What makes prions so weird is that they are a protein that has been mismade (misfolded, generally). That something is being made broken isn't that weird in biology. Mostly, the body degrades them pretty quickly. However, prions, while not being able to do what they're meant to do, can take well-made versions of itself and break them in the same manner.
And science doesn't know how they get misfolded in the first place then they basically go "hey other protein, boop you're like me now, let's burn this mother to the ground" and then you get something similar to the 'corrupted blood incident' https://en.wikipedia.org/wiki/Corrupted_Blood_incident except you lose your mind and die.
As far as saying we know how proteins misfold, I'm going purely by what I've read both on and on wikipedia as I'm not an expert in proteins and amino acids...
>It is not known what causes the normal protein to misfold
Second sentence of: https://en.wikipedia.org/wiki/Prion
The similarities to the 'corrupted blood incident' start and end with them both spreading to others in a local area. Prions don't really suggest that life is a simulation.
If life is a simulation, prions suggest either the simulation is run at an extremely high degree of verisimilitude, indeed simulating QM at a particle level for living things, or that the simulator has an incredible desire for accuracy, and has put a lot of non-trivial work into ensuring it. Almost anything you can think of that would reduce the computational load for simulating a human being would eliminate the possibility of prions arising in the first place, since "protein folding" is one of the first things you'd want to get rid of to cut the load down.
These are both somewhat my logic for even believing we live in a simulation. Obviously humans seem to be the largest factor on Earth given how we are rapidly changing the planet and directly, and indirectly, affecting all life on the planet.
We already have things like folding@home, weather modelling, climate modelling, asteroids@home, cosmology simulations from universe sandbox to supercomputer level, etc etc so on and so forth.
People love to immediately dismiss a simulation hypothesis "stuff is too detailed!" "why would someone use such specificity!". Well, why would anyone watch the Kardashians or eat hair (Trichophagia) or smoke cigarettes because none of these things are rational... however having a highly detailed simulation has all sorts of potential applications from seeing how ancestors may have evolved to seeing how a designer organism might potentially play out on a sterile world you plan to seed to being someone's incredible version of the Sims.
I mean, WE have the desire to do this sort of stuff. The above mentioned distributed computing and supercomputer applications shows some level of desire for this sort of thing but then you have games like SimLife from 1992 that allowed players to simulate an ecosystem and to modify the genetics of the plants and animals in the game.
'Junk' DNA, prions, viruses, erm consciousness, burning fossil fuels to the tune of roughly 40 gigatons of carbon dioxide emissions this year, living in coastal cities at or below sea level even AFTER disasters do considerable damage, the rapid adoption of social media and reality television, all of these things seriously make me wonder if we in fact are some sort of simulation just to see how a species can deal with both environmental and self-caused disasters.
Throw in many other things like the fact we've not detected life off-world, we've yet to detect any signs of another intelligent species (mind you we're just starting to really look), the rapid advances we've made in technology just in the 33 years of my life, technology allowing for us to fake audio and video of people that enough reference data exists for and things like this Chinese virtual news anchor, etc and it becomes really easy to go "huh, maybe we DO live in a simulation".
I do consider the simulation hypothesis to be one of the answers to the Fermi paradox, because "not deeply simulating the observable universe" is definitely another one of those obvious ways of cutting the load exponentially. Again, lots of attention to detail; if the external universe is something other than a full QM-level (or true ToE-level) simulation, it's obviously running on one heck of a detailed model... but it could still be exponentially less detailed than it superficially seems to be.
Of course, even if we are an island of advanced simulation, there could be others out there, surrounded by much-lower-detail areas.
Here, let me ask the most important question: What evidence about how things work on earth would make you less likely to believe the world is a simulation? Because it's always possible to come up with a justification after seeing evidence.
(Other planets are a different issue entirely.)
At the moment, reality is indistinguishable from being "real", for appropriate values of "real". There are things that would increase the likelihood of us being in a simulation, like us finding a QM effect of some sort where we can prove that what's actually happening is a computationally tractable simplification of what the theory presents. Instead, in the real world, every torture case we put QM to, QM keeps on QM'in just fine, regardless of what our intuitions think.
So I would say that for me, the simulation hypothesis is more-or-less as improbable as it can get. However, that isn't zero, and I can't see how it would ever be zero, and I have to admit that the "not zero" in question is not merely a pro forma "not zero, but 10^-huge number", but some sort of non-trivial "not zero". No idea how to put a precise number on it, though.
(As Scott Aaronson has said, one interesting reason to pursue quantum computers is that they are the things that will allow us to run experiments to see if reality does perhaps give up after a certain amount of entanglement and turn the system classical again despite there being no QM reason to do so. It's probably not the best reason to do it, but it is a reason to do it. If quantum computers turn out to be truly impossible, and not just an extremely hard engineering challenge, the reasons why can't help but teach us something profound about the universe.)
I'm not talking about absolute proof.
I'm saying that for a claim that evidence makes it "more likely", you can't just come up with a plausible reason. You have to weigh plausible reasons for and against it.
For every "more likely", there has to be a corresponding "less likely".
I can come up with plausible reasons that "less complex world" implies simulation, and I can come up with plausible reasons that "more complex world" implies simulation.
But unless I'm using conspiracy theorist logic, I have to weigh those against each other, and say that one of those pieces of evidence would overall make me less likely to believe in a simulation.
No, I'm saying that the fact that we can see down to a subatomic level which adds considerable complexity to a hypothetical simulation is not evidence that it is impossible that we live in a simulation.
If you tried to describe a 2018 smartphone to Konrad Zuse in 1936 he'd have thought you were either quite mad or a science fiction author. To him it would almost certainly be unfathomable as reality, even with him knowing that he created the first programmable computer. Hell, if you actually showed an iPhone to most of us in the 70's, 80's, or early 90's we'd likely have had a rough time processing it at first, especially if you loaded a VR game or some 4k video.
Okay, but I suggest you be a bit more careful with wording in the future. "These are both somewhat my logic" made it sound like the complexity was reinforcing the idea, rather than merely not completely disproving the idea.
With that clarified the rest of what I said is useless and a waste of both our time.
Second sentence of: https://en.wikipedia.org/wiki/Prion
>Prions don't really suggest that life is a simulation.
To you, to me they do. I imagine with the logic you're using, nothing suggests we live in a simulation. I however recognize this as one of dozens of things that are like "hey wait a minute". No where in my replies have I stated "THIS IS IRREFUTABLE PROOF REALITY IS A LIE!" I said this is one of the things that makes me seriously consider the simulation hypothesis. One. Of many.
>The similarities to the 'corrupted blood incident' start and end with them both spreading to others in a local area.
One is an anomalous protein that causes other proteins it comes in contact with to become anomalous spreading out of control and ultimately killing the host the other was code meant to do a specific thing, in a specific area, that due to a bug (that allowed the infection to leave restricted areas) caused anything that it came in contact with to become corrupted spreading out of control.
I didn't say "ermagerd this is the thing that happened on gorkamorka" I said 'this reminds me of the corrupted blood incident', which it in fact does.
There are a bunch of things I think that are more indicative (though, all have other possible, plausible solutions) of the simulation hypothesis. Some of the reasoning behind reality being a hologram (which I am not 100% on understanding).
For the corrupted blood, I wouldn't call it a bug. There was no problem with the software, just a wayward pet.
Prions misfold in a way that induces other of the normal version to misfold in the same way and therefore spread.
On the plus side prions kills pretty quickly and so there is unlikely to be much underlying mutation in their form or function.
Protein folding is incredibly complicated but I could imagine enzymes which could snip the prion or another similar protein which induces it to fold back into the preferred state. I doubt we have the science to explicitly design such a thing, but you could expose prion molecules in bacteria and put them under evolutionary pressure and they might eventually develop something that disables/fixes the prion.
You would essentially align survival of the bacteria to disruption of the prion molecule.
This link has an image of the two versions
https://www.mayoclinic.org/diseases-conditions/creutzfeldt-j...
Think of "slap bracelets", those curved metal strips that spring around your wrist. If you push two straight ones together, nothing happens. But if you push a curled one against a straight one it will make the straight one curl up too. The curled slap bracelet is acting like a prion.
Wow, I've not heard someone mention those in probably 2 decades.
Neural proteins are very complicated, so they need other proteins to help them fold properly. One of these protein-folding-proteins (PRNP) can be mis-folded in such a way that it mis-folds other proteins in the same way that it is mis-folded itself.
When most factory robots break, they simply stop working and they get recycled. But when a robot-making robot breaks in just the right way, it starts producing robot-making-robots that are broken just like itself.
It was an analogy and reading https://en.wikipedia.org/wiki/Proteopathy#Seeded_induction it sounds very much like they act like a piece of code corrupting another piece of code
>Some proteins can be induced to form abnormal assemblies by exposure to the same (or similar) protein that has folded into a disease-causing conformation, a process called 'seeding' or 'permissive templating'
That sounds a lot like many computer viruses
>A computer virus is a type of malicious software that, when executed, replicates itself by modifying other computer programs and inserting its own code
Prions are proteins too. And they can create themselves from source material too. But their source material are proteins which are not intended for processing. As such, prions are defined like weeds in a garden: They're not supposed to be there.
Marvel at the wonderful world that is biology: https://en.wikipedia.org/wiki/Protein