Eli5: What causes prion diseases, and why are they so hard to treat?

204 views

Like CJD, why haven’t we developed treatments so the disease isn’t a death sentence?

In: 3

4 Answers

Anonymous 0 Comments

Prions are a type of protein present in the nervpus system of some animals (the most famous are humans, cows and sheep) which has two different forms (or configurations): the normal one, and the infectious one (the prion itself). Prions can change the form of nearby proteins, which stop being functioning, producing mental illnesses such aa mad cow disease, scrapie in sheeps, and CJD in humans.
Mad cow disease became famous around 2000 irc because cow remains containing prions were being used to feed other cows infecting them. But other forms, like CJD and Scrapie, are genetic, so the only way of preventing them is via breeding.
The problem with treating the illness itself is that, like Alzheimer’s, its degenerative, so it destroys tissue and structures wich cannot br regenerated.

Anonymous 0 Comments

This previous post will be of interest to you :

Comment
byu/SqueakyFarts99 from discussion
inexplainlikeimfive

Anonymous 0 Comments

Proteins are like beads on a ball of string. They’re all tangled up, but their specific shape allows them to do what they need to do. If they’re out of shape even a tiny bit they don’t work and usually get broken down for their parts to build another protein.

Prions are proteins that are very toxic to the body in the way they’re shaped. However they also have the ability to take your normal protein with the same sequence of beads (amino acids) as it and when they bump into each other turn the normal one into the toxic shape. Even if your body is destroying the prions, which sometimes they struggle with, this bumping into the normal protein to make a new prion protein means it usually outpaces your body’s ability to deal with it

Anonymous 0 Comments

Prions basically self-replicate by changing the shape of other proteins, and they’re also really hard to deal with because prions are really, really small. They’re smaller than a virus, to put things into perspective. The immune systems struggles to “latch on” to something that small, which makes finding and destroying prions difficult. It’s easy for the prion to keep self-replicating while the immune system only slowly destroys the prions that it can find, which means that replication of the prion can out-pace the destruction, and the prion goes out of control.