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Quick notes on Prions

I don' recall hearing the word “prion” until I came across this tweet back in May of this year. I have no idea what it means, but it sounds bad. So where to start?

What are prions?

Prions are misfolded proteins that can cause brain disease, such as Creutzfeldt-Jakob disease (CJD) and Bovine spongiform encephalopathy (BSE, or Mad Cow’s Disease). They are not living organisms and do not contain genetic material, are not viral, bacterial, or fungal, but they are infections in that they can cause normal proteins they come in contact with to behave abnormally.

Where do they come from?

Prions occur naturally when normal proteins malfunction, and can be accumulated by coming into contact or by ingestion. What caught my attention initially was how the Tweet mentioned prions could contaminate the ground, infecting the grass and any wildlife that feeds on it.

How can you get rid of prions?

With much difficulty, it seems. Prions are resistant to conventional methods of decontamination or sterilization. They may even persist on surgical equipment which undergo rigorous sterilization procedures. The best method is by cleansing with high temperatures combined with chemical solutions. Still, this does not eliminate them entirely, but only decreases the likelihood of infection.

There was a reply to the OP that mentioned that an enzyme called prionzyme has been developed that can help limit the spread. However, it is still not widely available.

How much exposure is a problem?

Prions have higher concentration in certain organs and tissues, such as the brain, lymph nodes, and spinal cord. Ingestion or coming into physical contact can result in infection, which unfortunately has a long incubation period that is measured in years/decades.

Given how hard they are to remove and how dangerous they can be in small amounts, current practices focus on preventing prions from entering the food supply.

What measures are taken to keep them out of food?

Surveillance of livestock and testing animals for prion infection is the main approach. Bans on animal feed that contain the organic material that can be most infections (brain, spine, etc.) are in place to ensure outbreaks such as BSE in the UK during the 1980s / 90s do not recur. Traceability and information on the country of origin is also used to ensure standards are observed in import/export across countries.

What about other infection routes?

Other than contaminated food, blood transfusion is another method by which prions infection can spread. There is no test that can be done to screen donors for potential infection. Eligibility deferrals for travel/residence in the UK during the 1980s has been removed for the US, but still in effect for other countries such as Japan.

Takeaways

Thus concludes my crash course in self education about prions. This is haunting stuff. Forget about salting the earth. What happens if your farm land is peppered with prions and you did not know about it? What if it's not you, but the farmers that supply beef to the burger restaurants throughout the world?

In an age where your burger could contain the meat of up to a hundred cows, prions sound like an absolute nightmare. What's more, it feels like there is realistically very little I could do to eliminate the threat. What can you do against an invisible protein that can cause disease decades from first exposure? A substance that is highly resistant to sterilization? I have no idea (yet).

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