: Why do prion diseases have 100% fatality rate ?

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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

18 Answers

Anonymous 0 Comments

Prions are misfolded proteins. They cause other proteins they come in contact with to misfold as well. They are kind of like zombies in that regard. A misfolded protein is no longer able to do its job in the body. Once too many misfolded, you get sick and eventually break down and die.

The only way to destroy them is with extreme heat, which naturally can’t be done while the person is alive.as it is just a protein the immune system has no way of dealing with them. It doesn’t recognize them as a problem.

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