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I did a bunch of research (googling) back in the late 90's or 2000s when the mad cow disease outbreak first happened mainly in England. I thought it was pretty terrifying and I wanted to know what the hell was going on. I read about Kuru all the way back then and the take away was that people shouldn't eat the brain and nervous tissue of other people or Kuru could result. But also it seemed that mad cow disease was occurring because cows were being fed the ground up remains of other cows and that included cow brain and cow nervous tissue. So basically what was happening with cows was the same as what was happening with people getting Kuru.
I also recall learning that in order to get mad cow disease you would need to eat cow brain or cow nervous tissue. Of course, you would probably assume, as I did, that you are safe because you don't eat cow brains or nervous tissue. Well, you could be wrong about that. There is something called AMR (advanced meat recovery). As I understand it, when the meat processing plant removes the spine and skull from the cow there is still some meat stuck to the spine and skull. The AMR process entails blasting off that meat stuck to the spine and skull with some kind of high pressure for use in ground meat. But the result is that some nervous tissue from the cow can get mixed up in ground meat created with the AMR technique and that is one way that you could end up eating cow nervous tissue. After I did that research, I swore off ground meat.
No-meat diet is not 100% prion-proof according to Wikipedia:
> 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.
True, but one might argue that it's at least a step in the right direction if you're really worried about transmissible prions.
Prions are nature's grey goo.
The Wikipedia article on Kuru mentions some people developing immunity to it in the most affected areas. Very interesting.
In 2009, researchers at the Medical Research Council discovered a naturally occurring variant of a prion protein in a population from Papua New Guinea that confers strong resistance to kuru. In the study, which began in 1996,[21] researchers assessed over 3,000 people from the affected and surrounding Eastern Highland populations, and identified a variation in the prion protein G127.[22] G127 polymorphism is the result of a missense mutation, and is highly geographically restricted to regions where the kuru epidemic was the most widespread. Researchers believe that the PrnP variant occurred very recently, estimating that the most recent common ancestor lived 10 generations ago.[22][23]
Of the discovery, Professor John Collinge, director of the MRC’s Prion Unit at University College London, has stated that:
It's absolutely fascinating to see Darwinian principles at work here. This community of people has developed their own biologically unique response to a truly terrible epidemic. The fact that this genetic evolution has happened in a matter of decades is remarkable.
— John Collinge, Medical Research Council The findings of the study could help researchers better understand and develop treatments for other related prion diseases, such as Creutzfeldt-Jakob disease[21] and Alzheimer’s disease.[24]
A workaround for people who would like to eat ground meat but avoid this issue is to grind it yourself, or to buy steak and have the butcher grind it for you.
"Beef brains and veal (juvenile beef) or calf's brains are used in the cuisines of France; Italy; Spain; El Salvador; Mexico, etc. where they are called sesos in Spanish and are eaten in tacos and quesadillas; Pakistan, where they are known in Urdu as Maghaz; Portugal; Indonesia; and in the United States, especially in St. Louis, Missouri, and the Ohio River valley."
People do eat cow brains...I have at regular ol' restaurants in the USA.
> I did a bunch of research (googling) back in the late 90's or 2000s when the mad cow disease outbreak first happened mainly in England.
This is not directly related to the main thrust of your comment, but I thought it was interesting that more than 80% of the BCE cases in England occurred in the years 1990--1995, when Google did not exist in any form. Only 6--7% of the BCE cases in England occurred after google.com was registered.
> The AMR process entails blasting off that meat stuck to the spine and skull with some kind of high pressure for use in ground meat.
This is true. A while back, I read stories of how (Mostly low-wage Mexican) Workers at Meat Plants were getting a strange neurological disease that confounded their doctors. The cause was then traced to them inhaling the aerosol of the Animals' brains being blown out, mostly Pigs.
Related News / Source:
- The Spam Factory's Dirty Secret => http://www.motherjones.com/politics/2011/06/hormel-spam-pig-...
- Vaporized Pig Brains Are Torturing Austin's Migrant Meatpackers => http://www.citypages.com/news/book-vaporized-pig-brains-are-...
AMR and such, is that our famous pink slime that became all the rage/outrage awhile back? To the point we have quite a few establishments making it well known they never used such products.
industrialization did not do any favors towards animals, especially in regards to processing and such for food
I first heard about prions and Creutzfeldt-Jakob Disease after getting a letter saying I may have been exposed.
I had surgery after someone with CJD, but before that person died, was autopsied, and diagnosed.
That was over a decade ago. So far no signs of it. I did make peace with the fact that I have a much higher probability of dying from it than the average American. The only substantivu changes in my life are:
(a) My family and I have had very frank discussion about death earlier than we would have otherwise.
(b) I no longer donate blood.
(c) I removed myself as an organ donor. Although my family knows I still want my body to be available for science.
>> My family and I have had very frank discussion about death earlier than we would have otherwise.
It would benefit any family to have that discussion, even if both parents are in their 20s and there has been no "scare scenario". Many people die every single day due to unpredictable events, with no warning whatsoever. Unfortunately, too many people have never really pondered their mortality, leading them to be incapable of even considering what it would mean if man/woman/child died tomorrow. Having a sit-down meeting is likely to be brushed off as a "meh, it'll never actually happen to us".
Any one of us could be dead in the next 5 minutes. That's more reality than paranoia. The trick is to get to the point where you understand just how fleeting life is (you or a loved one might be gone any day now), but without letting it negatively control your life.
>> I did make peace with the fact that I have a much higher probability of dying from it than the average American
You should stop thinking that way. A decade past the initial scare, you're likely no more at risk than anyone else. You weren't given the disease, you were exposed to a small possibility of it. It's still the responsible thing to not donate blood or be an organ donor, but as far as your day-to-day life goes you should consider yourself squarely in the "average" category.
Wow, what a frightening letter to receive! Thank you for sharing and best of health to you and your family.
Shouldn't autoclaves destroy all proteins, prion or otherwise? What exactly is the exposure vector?
edit: Unsourced statement from wikipedia[1]: "However, prions, such as those associated with Creutzfeldt–Jakob disease, may not be destroyed by autoclaving at the typical 134 °C for three minutes or 121 °C for 15 minutes.[citation needed]"
I understand prion diseases are rare and it's more likely I'll die in a car accident or something, but the persistence of prions is just a horrifying concept.
Highly resistant to heat, radiation, proteases, enzymes... Basically if you've got a prion disease it's just a matter of time before they end you.
They really are terrifying. It's hard to say exactly why this is so, but I think it has something to do with how they can occur (some protein randomly misfolding) and that they aren't really "alive", in the same sense that viruses and bacteria are.
You can kinda forgive viruses and bacteria: they're just looking to survive and multiply, just like us. In some cases they monopolise so much of their hosts' resources, or some really critical resource, that the host dies. But Prions, uhhhhh.... They're just kinda these unnatural things that go wrong and then kill you in pretty awful ways. Though not because they necessarily need to out-compete you for resources. Indeed, they don't seem to have any innate preference for existing or not existing, or thriving or not thriving. But they kill you anyway....
Also, just to scare you a little more, one of the more popular (perhaps dominant) theories about Alzheimer-family diseases is that they are initially caused by Prions, that migrate into your brain and start forming amaloid plaques, and then it's all down-hill from there.
On the upside, I vaguely remember reading some research on a 'almost ready' drug, initially developed to lessen the negative side-effects of chemo-therapy, that might be effective at stopping the progression of these plaques as they spread throughout the brain. If it actually works, we might have a treatment of AD-family diseases on the market in a year or so (the chemo folks have already gone through the lengthy human safety testing part of the exercise).
Not a cure, but at least it might halt or significantly slow progression of symptoms.
I'll probably pass on the Elk jerky on my next road trip through SW Colorado.
What would a cure for prion diseases even look like? Are prions targeted by the immune system at all? That to me is what is scary about them.
It can happen anywhere at anytime, that's why it's so terrifying.
> Basically if you've got a prion disease it's just a matter of time before they end you.
Unless you had a mind backup and a second body ready.
Which, I think, all medicine ultimately aims, should aim, for; Why bother learning how to repair the single same body over and over (which won't help in unexpected catastrophic failures aka fatal accidents anyway) when you can just grow and jump into a new one?
Many of you have probably heard about Prions and Papua New Guinea but the end of the article discusses new prion diseases showing up in deer within North America.
The idea that human's could get a prion disease from deer meat, coupled with a potentially long incubation period, is frightening. Living in a hunting community I may avoid eating deer for the foreseeable future.
it's highly unlikely... There's a (probably directed, weighted) graph of species-species transmission potential, and it does seem to obey some rough distance rule, which does NOT correlate necessarily with species distance rule. We think that cows can transmit to humans; cows and sheep can co-transmit. (if you have a sheep with scrapie sharing a pasture with cow, the cow can get mad cow disease). deer though do not seem to transmit to cow, even though CWD is super common in deer and deer and cow pasture together pretty much everywhere in the US except maybe hawaii.
Here, a transgenic mouse with human prion protein is resistant to CWD transmission; but the same mouse with elk protein is susceptible.
http://www.tandfonline.com/doi/full/10.1080/19336896.2015.11...
Reminds me of a scene in the film Book of Eli, which, if you're a fan of Fallout, should watch.
An underrated movie, imo.
Prions were the cause of the BSE cattle disease. more commonly known as mad cow disease in europe some years ago, which was blamed on cattle being fed animal products. It killed a couple hundred human and millions of animals either died or were slaughtered.
The incubation period is several years so at the height of the crisis the worst case scenario involved hundreds of thousand of people developing the disease.
https://en.wikipedia.org/wiki/Bovine_spongiform_encephalopat...
Lots of US people end up hearing about this in a roundabout way, having lived in the UK for a period of time is a disqualifier for donating blood in the US. So each time you donate blood you read a question asking about cumulative time spent:
https://www.fda.gov/BiologicsBloodVaccines/BloodBloodProduct...
Can we view this as nature's mechanism to prevent cannibalism amongst species? Mad cow disease occurred when cattle were fed the remains of other cattle in the form of meat and bone meal
I think assigning animus to nature is misleading here. It's a factor that once known would influence people away from cannibalism, but there's plenty of cannibalistic species that don't seem to suffer from prion diseases (and CWD is the opposite -- prion disease with rare cannibalism).
Why would nature care?
If anything, the aversion to cannibalism would be an effect of prion disease.
Interesting side story is that Gadjusek did a year in prison for child molestation (which he think is a natural/good thing).
There is a documentary on him on BBC called the Genius and the boys https://www.youtube.com/watch?v=4OxppDxzSww
Researcher: "I have no solid idea where this problem could be coming from. As a last resort I'll examine whether the ritualistic consumption of decomposing human brains may have something to do with it"
> Lindenbaum had a hunch about what was going on, and she turned out to be right. It had to do with funerals. [0]: Ibid.
Can you be tested for prion disease?
Indeed, you can. What you cannot do, however, is remain alive to get the results.
The last I heard the test can only be done during autopsy. (Because they're analyzing slices of brain tissue.)
For the past months I've been seriously considering starting a theme restaurant that served wild meats - deer, hogs, gators, large constrictors, etc. That idea just went out the window.
Prion-disease transmission from wild game is still quite rare.
I'd be more worried about exotic parasites or radioactivity from the wild hogs.
[0] http://www.telegraph.co.uk/news/worldnews/europe/germany/110...
Around here, deer meat is illegal to sell. You would probably have to farm the meats.
>My own interest in this question became rekindled by my experiences in New Guinea over the last 50 years. When I arrived in New Guinea for the first time, it became clear to me almost immediately that New Guineans are curious, questioning, talkative people with complex languages and social relationships, on the average at least as intelligent as Europeans and Americans.
http://www.jareddiamond.org/Jared_Diamond/Guns,_Germs,_and_S...
Is it certain this came from cannibalism and not infected pigs?
Yes, very certainly. When the cannibalism stopped, so did instances of Kuru. Although you can get Prion diseases from eating infected pig brain and nervous tissue, the instances discussed in the article were solely caused by ritual cannibalism.
This article make me unwell. Just thinking of the cannibalism makes me wanna throw up.
Let me accelerate that then:
And that's why you never grok.
NPR needs to be more concise. Their informative articles such as this one are written as if the writer is trying to get an A in creative writing 101. Knock off the color and give us the scoop in 2-3 paragraphs.
Okay, atleast some benefit of being a vegetarian. But iirc there was some vegies too that can infect the brain.