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by gustavus 822 days ago
So that has raised an interesting question in my mind. But as far as I know the only significant case of prion disease amongst human beings, excluding CJD which seems to be generically determined, was the instance of Kuru among the Papau New Guinea tribes; however we know other societies practiced cannibalism at a certain scale such as the Aztecs in which people were eating other people all over the place, yet we don't have widespread evidence of Kuru.

Is there something distinct about Brian tissue which puts it at a higher risk for prion disease formation?

2 comments

Just a bystander shooting from the hip, but does the fact that brain nerve cells aren't recycled and rebuilt but live as long as the person does mean they are more prone to accumulating bad proteins?

Other tissue like skin or muscle that regularly break down and regrow would offer a lot more opportunity for the body to filter out the prions, but once in the brain cells, there's nothing for them to do but accumulate.

It's because the prion protein is in the brain. As in, there is basically only one protein in mammals which actually has this problem (that we know of), it's not a general issue with proteins. The variants which exist are essentially because of the small variations in this protein across species, or specific inheritable mutations which make sponteneous formation of the misfolded form more likely.

(this protein, by the way, is not essential for life, but lacking it seems to slow brain development in mice)