: 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

A prion disease is an incorrect fold in your brain proteins (needed for your brain to survive) – it also unfortunately a more ‘optimal’ fold in a lot of cases, so it ‘corrects’ the other proteins in your brain to be folded wrong and clump together with it. Each wrong protein also has the potential to cause a chain reaction and make thousands more wrong proteins, which makes it impossible to cure even if you can remove one clump.

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