What exactly are prion diseases and how do people get them?

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What exactly are prion diseases and how do people get them?

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Prions are weird. In short, they’re a kind of protein that can warp other proteins into prions. This makes them Extremely dangerous as they’ll slowly assimilate every protein in the body starting with the brain. They’re also unusually durable and can’t be treated with the same medicines you’d use to fight bacteria or virii.

While it depends on the prion, the most well known vector is eating. Mad Cow disease was introduced to humanity when someone ate a steak from an infected cow.

Prions are proteins that have gotten misfolded…we don’t know how, or why, but we do know that one misfolded protein can influence another to misfold, then another etc, etc…. This is how prion diseases spread.

Proteins start out as something called amino acids, which are strung together in a chain, like a beaded necklace. And similar to if you twist a beaded necklace around in your hand, it starts to coil up on itself, these amino acid chains fold in predictable ways to form proteins, which perform various functions. If a protein isn’t folded in the correct way, it won’t be able to interact with the other proteins that it’s supposed to, halting many of the bodies essential processes.

Prion diseases seem to arise due to cannibalism. It’s been observed across multiple mammalian species, including cows (Creutzfeld-Jakob aka Mad Cow), sheep (scrapies) and humans (Kuru). Notably with CJD, when cow feed has bovine bone meal in it, the cow can develop CJD, which then can pass to humans who consume the infected meat.

Prion diseases are mis-folded proteins (usually left-handed folds). They can replace or displace right-handed proteins, and they don’t “work” the same way as the proteins they displace. That causes all sorts of weird stuff to happen in the body, and we can’t (yet) even *make* left-handed proteins in a lab, so it’s very, very hard to study them and develop real cures. Mostly, we just have treatments (some more effective than others)

At least, that’s my understanding of it. I could be way off, I’m not a molecular biologist

This Podcast Will Kill You has a great episode on Mad Cow and discusses prions pretty well:

https://podcasts.apple.com/us/podcast/this-podcast-will-kill-you/id1299915173?i=1000430171031

A prion is like bad code on a computer. This bad code gets in the way of other processes and causes cascading problems. Whereas a virus seeks out to replicate itself, a prion is simply a mishshappen protein that damages other protiens and gets in the way. The body needs these protiens to regulate itself so slowly but surely the prion will break down vital functions of the body.

Now prion diseases are mostly rare because they usually require consuming the protien directly. They can’t spread on the air like a bacteria or virus might. One of the reasons we had such a problem with mad cow disease was that animal feed was repurposing the unused organs. This included the brain and central nervous system that the prions was in which ended up spreading the prion to other cows. Often times the meat from infected cows was safe to eat as the prion couldn’t leave the brain. However if a blade or surface had the prion on it from passing through infected nervous tissue of the cow then it could be spread onto the meat. So we infected a bunch of cows on accident and couldn’t risk the possibility of it getting on the meat.

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What exactly are prion diseases and how do people get them?

In: 1

Prions are weird. In short, they’re a kind of protein that can warp other proteins into prions. This makes them Extremely dangerous as they’ll slowly assimilate every protein in the body starting with the brain. They’re also unusually durable and can’t be treated with the same medicines you’d use to fight bacteria or virii.

While it depends on the prion, the most well known vector is eating. Mad Cow disease was introduced to humanity when someone ate a steak from an infected cow.

Prions are proteins that have gotten misfolded…we don’t know how, or why, but we do know that one misfolded protein can influence another to misfold, then another etc, etc…. This is how prion diseases spread.

Proteins start out as something called amino acids, which are strung together in a chain, like a beaded necklace. And similar to if you twist a beaded necklace around in your hand, it starts to coil up on itself, these amino acid chains fold in predictable ways to form proteins, which perform various functions. If a protein isn’t folded in the correct way, it won’t be able to interact with the other proteins that it’s supposed to, halting many of the bodies essential processes.

Prion diseases seem to arise due to cannibalism. It’s been observed across multiple mammalian species, including cows (Creutzfeld-Jakob aka Mad Cow), sheep (scrapies) and humans (Kuru). Notably with CJD, when cow feed has bovine bone meal in it, the cow can develop CJD, which then can pass to humans who consume the infected meat.

Prion diseases are mis-folded proteins (usually left-handed folds). They can replace or displace right-handed proteins, and they don’t “work” the same way as the proteins they displace. That causes all sorts of weird stuff to happen in the body, and we can’t (yet) even *make* left-handed proteins in a lab, so it’s very, very hard to study them and develop real cures. Mostly, we just have treatments (some more effective than others)

At least, that’s my understanding of it. I could be way off, I’m not a molecular biologist

This Podcast Will Kill You has a great episode on Mad Cow and discusses prions pretty well:

https://podcasts.apple.com/us/podcast/this-podcast-will-kill-you/id1299915173?i=1000430171031

A prion is like bad code on a computer. This bad code gets in the way of other processes and causes cascading problems. Whereas a virus seeks out to replicate itself, a prion is simply a mishshappen protein that damages other protiens and gets in the way. The body needs these protiens to regulate itself so slowly but surely the prion will break down vital functions of the body.

Now prion diseases are mostly rare because they usually require consuming the protien directly. They can’t spread on the air like a bacteria or virus might. One of the reasons we had such a problem with mad cow disease was that animal feed was repurposing the unused organs. This included the brain and central nervous system that the prions was in which ended up spreading the prion to other cows. Often times the meat from infected cows was safe to eat as the prion couldn’t leave the brain. However if a blade or surface had the prion on it from passing through infected nervous tissue of the cow then it could be spread onto the meat. So we infected a bunch of cows on accident and couldn’t risk the possibility of it getting on the meat.