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
Normal prion proteins’ function in the brain is not nailed down, but it essentially protects neurons from damage such as oxidative stress and excess ions like copper. It’s not really a single protein, but a complex of proteins and a couple other anchoring molecules. Since there are many molecules involved, if it is damaged and folds the wrong way these broken, unstable molecules will bond to anything, preventing the complex from returning to it’s original structure. The instability of the broken molecule affects those around it, as there is a delicate balance to maintaining chemical bonds within the brain. Without prions, neurons are susceptible to apoptosis, or programmed cell death, and synaptic degeneration, resulting in cells not being able to communicate. It loses it’s delicate connections and dies from the inside out.
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