I wonder if it slowly progresses over time, or if it develops opportunistically once some other bodily system that keeps it in check breaks down with age.
You have one misfolded protein that “teaches” other proteins to misfold in the same way[1]. It’s very slow initially because you’re talking about individual proteins, and it takes a while for each prion to bump into a normal protein of the correct type. The immune system can’t do anything about them though, so once a protein is converted there’s permanently another prion. The conversion rate is very slow, but it never ever ratchets back - the number only increases. It’s a geometric progression.
Once symptoms show up there’s a TON of them, and it goes downhill fairly quickly. That’s why there’s such a long latency period.
Once symptoms show up there’s a TON of them, and it goes downhill fairly quickly. That’s why there’s such a long latency period.
[1]It’s like Ice-9, if you’ve read Cat’s Cradle.