Prions! The Proteins that Eat YOU!

You fucking love SCIENCE!!!

Protein. You need to eat it. Even my vegetarian daughter gets it from beans and other non-cute sources. Your body is made of it. Well, it might be more fat than protein these days, since we've all gained 25 pounds under Trump, right? But you know what I mean. Before we get to the scary part of this article, a quick primer on protein. What is it? How is it made? I'm glad you asked!

Your DNA codes for all the proteins that your body makes. The DNA chain is made of nucleotides (think Lego blocks) that are the blueprint for how amino acids (protein Lego blocks) get linked up further downstream on the way to make a protein. After that there is other biochem magic that happens (quick summary here for those who forgot high school biology) and you end up with a nice, shiny protein.

Some proteins do work as enzymes, some work as tiny tunnels in your cells to let molecules in and out in an orderly manner, and some puto proteins become prions, which are basically tiny Jeffrey Dahmers . They want to eat your brain. Straight up. No chianti or fava beans (yeah, I know that partners better with liver) for those tasteless pendejos.

Proteins that become prions are a result of a mutation/biochem fuck-up along the way from DNA blueprint to the final version sitting in the protein showroom with undercoat and ski rack. Proteins are three dimensional thingees that need to be in the proper shape or "folding" to do their job. Prions, because the amino sequence is wrong for the intended protein, "misfold" and take on a different shape than intended. Sometimes, when proteins misfold, enzymes will just chew them up and take care of the problem. Not so much with prions. These fuckers misfold in such a way that they become a type of infectious molecule and cause OTHER normal proteins to misfold.

Now, you probably know prion handiwork best from the peak hysteria of mad cow disease, or bovine spongiform encephalopathy (BSE). This started in the late '80s in the UK, and led to mass cow slaughters, new regulations and some human deaths. This is also why I can't buy tacos de cesos here in the states anymore. Speculation is that feeding British cattle the remains of other dead cattle and sheep already infected with prions spread BSE across the land. This was the first time there was wide knowledge of prions and what havoc they can cause, but the first time it was seen was in 1732 in "scrapie," a disease of sheep and goats. The name comes from the fact that most infected animals try to scrape off their fleece using trees, rocks or whatever they find due to a crazy itch. Other signs include excessive lip smacking and a fucked up gait. Until we can test the Orange in the White House, we can't know if this great American nightmare isn't just due to prions in his Big Macs.

BSE and Scrapie are definitely scary, but my favorite prion disease has GOT to be Kuru (not to be confused with " the world's most comfortable shoes "). It's also known as "laughing sickness" because in addition to other symptoms, you laugh like a crazy person when you get it. This is the realm of horror movies. The Fore people of Papua used to cook and eat their dead as part of a funeral ritual to help the soul be free of the body. Don't laugh if you're a Catholic. Sadly, women and children would eat the cooked brain, which has the greatest concentration of prions, so would succumb to the disease more often than adult males.

More worrisome for those of us in North America is the Chronic Wasting Disease in deer, elk and other related wild animals. It's been spreading for years now and while there have been no human cases related to it, if you're a deer hunter, don't kill and eat deer that seem too thin or lethargic. That's the first hunting advice I've ever given!

 "And now, for something completely different!" 

So, all this is interesting stuff (I hope!) but the current events angle is even more interesting. Remember two paragraphs ago and Kuru? Well, how did that outbreak start? This takes us back to the beginning of the article and how proteins are made. Speculation is that some member of the Fore tribe developed the prion disease, died and then spread it to the rest via the funeral Golden Corral-fest. That patient zero had a mutation in his/her DNA that then led to a misfolded bunch of proteins/prions. Of course, that occurred before all the genomic advances we have today where you can sequence someone's DNA in 24 hours.

In 2011, Sonia Vallabh, post-law school and working on city infrastructure, got some genetic testing done. Her mother had died of a rare disease, fatal familial insomnia. Besides the obvious problems with sleep, there is also dementia and other symptoms. The cause? You got it. Prions. Sonia tested positive for the disease, meaning that she would absolutely develop it and die from it. What was her response to the dire news? To flip off the pendejo proteins and switch careers! She and her husband (and engineer) went back to school to become PhDs (graduating next spring!) to become experts in the field and work on a cure for the super rare disease and save her life. Of course, if they pull it off, not only will I be in line to option the screenplay rights, but they will crack a medical puzzle that has eluded top minds since the 1700s! I am 100% rooting for them and will be following their story.

Here are a couple of more detailed versions of their story. Linkee 1 and Linkee 2 .

Now it is your OPEN THREAD! Don't forget to tip your Wonkette so we NEVER DIE.