I recently found out about the so-called prion diseases, which are incurable and fatal diseases that affect (a euphemism, the real word is destroy) the CNS and cause a rapid deterioration of mental and physical abilities.
There are many prion diseases, the two most famous are probably the mad cow disease (non-human mammals), and the Creutzfeldt–Jakob disease (CJD) (for human mammals). Both are 100% fatal, and *no one* is known to have survived longer than 2.5 years after a CJD diagnosis. That’s the kind of stuff you wouldn’t wish on your worst enemy.
Why are these diseases so deadly? I read that it has to do with abnormal proteins but that was way over my head.
EDIT : I have another question, can prion diseases be rightly called the most dangerous diseases known to man ?
Thanks;
In: Biology
So proteins are almost always folded so that they can do stuff. When something happens like genetics or a disease, then you get these misfolded proteins. Sometimes, these are corrected. But when they are not, you get these prions.
Prion are misfolded proteins that misfold other proteins, which causes a sort of domino effect until you have so many misfolded proteins accumulating in the brain and that causes damage.
Now, your body doesn’t easily recognize these prions as foreign as easily as it would for bacteria, viruses, or fungi because these proteins are not foreign! They’re just mistakes of your body’s own proteins.
The reason why there is such a high fatality rate is because we have no current drugs to “kill” these misfolded proteins without killing your normal proteins. However, there is some research [some research](https://www.science.org/content/article/can-new-drugs-stop-deadly-set-brain-eating-diseases) into drugs that turn down expression of these proteins at the source. They prevent prions from being made. Hope this helps.
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