Eli5 what are prions and how do they misfold and cause diseases?
In: 0
A prion is a protein. All proteins rely on shape to do things, and the way they take this shape is by folding. The way it folds is determined by the structure as well as how it is made. And these proteins are what makes up everything in your body that does things. Most of the time this is fine, but if a protein gets folded incorrectly then bad things can happen. This is the misfold you are referring to. Most of the time it’s not an issue and it just doesn’t work. Sometimes though, this new fold is such a low energy fold that other proteins of the same type want to get in in the action and refold themselves to match it. That’s a prion. And like cancer it’s still you so your body doesn’t really have a way to identify and remove it. And because proteins make up you, eventually doing all this misfolding will cause significant issues and you go insane then die.
Proteins are very long molecules in your body that do various jobs. For instance, hemoglobin holds onto oxygen so that it can be transported around your body.
The shape and composition of a protein determines what it can do. Certain shapes are considered stable and useful. Many shapes are unstable, and degrade into a stable form or just disintegrate. Rather rarely, however, a protein will be formed in a wrong configuration that is stable. Even worse, that protein can make other proteins also malformed. The body doesn’t recognize proteins as bad, so it can’t recognize a problem, nor does it really have the tools to clean it up. The bad proteins build up, usually in the nervous system and killing cells which causes holes in brain tissue. Obviously, holes in your brain is not good, leading to all sorts of neurological issues and eventually death.
Latest Answers