: 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

They are misfolded proteins that cause other proteins to be misfolded. This is very dangerous to the brain because it disrupts the white and grey matter structures of the brain, slowly destroying it and eventually causing death. You can’t destroy them like bacteria or viruses because it’s just a bunch of structural molecules, not a living thing which can have its metabolism disrupted (bacteria) or be tagged by antibodies (viruses). Now they’re not invincible, but you need either a very strong denaturing agent or extremely high temperatures. Suffice to say that kills the person infected.

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