Prions, How Do They Work?

Also, what the hell are they? To be honest, before researching this blog entry, my image of a prion consisted of a small ball with a happy face on it that gave you mad-cow (as it turns out, I wasn’t too far off).

According to American Heritage Dictionary, a prion is “a microscopic protein particle similar to a virus but lacking nucleic acid, possibly the infectious agent responsible for scrapie and other degenerative diseases of the nervous system.” Thanks, the internet! Now I know exactly what a prion is! I am so happy about that.

But what does a prion do? For this answer, I turn to a more serious-faced resource, the Centers for Disease Control and Prevention (remember the CDC? It’s been mentioned in movies). The CDC doesn’t like prions. They say all sorts of bad things about them. This is probably because prions cause horrible, debilitating diseases that end in agonizing death. Here’s what they have to say about the matter:

A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

That sounds bad. It sounds bad because it is bad. Prions basically eat our brains. Here is the CDC’s list of diseases for which prions are thought to be responsible:

Creutzfeldt-Jakob Disease (CJD)

Variant Creutzfeldt-Jakob Disease (vCJD)

Gerstmann-Straussler-Scheinker Syndrome

Fatal Familial Insomnia

Kuru

Bovine Spongiform Encephalopathy (BSE)

Chronic Wasting Disease (CWD)

Scrapie

Transmissible mink encephalopathy

Feline spongiform encephalopathy

Ungulate spongiform encephalopathy

So, my original idea of a prion being a smiling ball that kills has been revised. A prion is an evil squiggly line that kills. Here is an artistic rendering using 3-D technology: