This doesn’t just happen to folks who get the prion from cows, many people in my area got sick and died after they ate prion infected squirrel brains.
Prion based illnesses are among the weirder, more poorly understood, nonsensical and scarier maladies out there. The idea that a “misfolded” protein can cause normally folded proteins to join it in rebellion and then spread to other bags of proteins is just bizarre. Even more “nonlife” than viruses, but seemingly more “infectious,” if that is even the right word for it.
A brief synopsis:
Prions are pretty scary, seems nearly unstoppable from what i know of them.
I’ve been suspicious of the sudden drought of Mad Cow Disease news back in the early 2000s.
I’m still waiting for a sudden “unexplained” outbreak, since these things take more than 10 years to take hold.
Username checks out.
In NB Canada, we are dealing with a mystery neurological disease that has symptoms similar to Creutzfeldt-Jakob disease. This is the first time I’ve ever read about this disease other than what’s happening here.
I think the best way to think of them is as a cascading bug in proteins. Not a pattern that wants to replicate, but a sort of creeping syntax error, a glitch that is inherent in all proteins that just needs a trigger to bring the system crashing down.
It was in the news in the early 2000s since that’s when people were dying of it, after consuming UK cows in the early 90s.
The UK killed all their cows and the US stopped feeding dead cows to live cows and that fixed the immediate problem. The fact that we almost never test cows for Mad Cow helps keep the number of alarming news stories to a minimum.
The (slim) prospect of contracting a prion disease is in some ways a better psychological motivation for me to cut down on my meat consumption than the moral and environmental arguments, which are strong in their own right.
I ate elk on a trip to the western US back in 2013. They are known to have problems with similar prion diseases on occasion. I guess sometime in the next 2 to 5 years I’ll know if I fucked up there.
I was in Italy in the mid 90’s with the Navy so now I am disqualified from ever donating blood due to the mad cow outbreak during that time.
Not sure that works. These things don’t affect the transcription or translation mechanisms, they seem to alter how proteins are folding by their simple presence. As I said, poorly understood, and the research is obviously hard to do and quite dangerous. Luckily, they are not easy to contract for most of us, although we are seeing a lot of Chronic Wasting Disease among deer here in Virginia, and I am pretty sure they are not eating each other’s brains. Does make a really good argument for Kosher beef, though!
Yikes. I feel like if I was around infections needles all day, I’d want prick proof gloves, if there is such a thing.
IIRC something similar happened with some highly toxic form of mercury that killed the lab worker months later.
It was everywhere in the news during the BSE crisis
Interesting, I am high-level familiar with Mad Cow/BSE, but was not familiar to its link to Creytzfeldt-Jakob, or prions for that matter. Learn something new every day.
Same. Sort of. I ate beef when I lived in London in the mid-80s. I gave over a gallon of blood in the late '80s and early '90s before my having lived and eaten beef in the UK became a blood donor disqualifier. Just did a quick check on the googler, and apparently they still don’t want my blood.
Prions are fascinating, but with 20+ years working in industrial and pharmaceutical labs, these stories always make me gulp and clench. So much lab work requires you to be on MAX ALERT MODE that it can be exhausting. I’ve had many close calls like having an aerosol can of hot adhesive burst and splatter across my safety goggles. I kept them on my bench as a reminder for many years. Stay safe out there, lab rats!
That accident crossed my mind as well.
In familial Creutzfeldt–Jakob disease and fatal familial insomnia the prions are produced by a genetic mutation so maybe the deers are suffering from a hereditary disease rather than a contagious disease.
Good memory. That was dimethylmercury poisoning. IIRC, the victim was double-gloved with latex gloves and ~2 drops of DMM fell onto her gloved hand. It turns out that it only takes around 15 sec for DMM to permeate through latex gloves, at which point it is directly absorbed through the skin. No needle stick required. It took around 3 months for symptoms to show up and she was clinically brain dead a few months later.
TLDR: organomercury is some seriously scary shit, but a little too slow-acting for Vlad’s tea service.