- the working hypothesis is that they’re misfolded proteins that catalyze the misfolding of healthy versions of the same protein, eventually causing plaques of misfolded protein to accumulate in the brain
- how do prion proteins get into the brain?
- mice with the normal version knocked out are not perfect, but too functional to elucidate what, exactly, it does
- prions are extremely hard to destroy (ionizing radiation doesn’t work, proteases don’t work, high temperature doesn’t work). a combination of high pressure, temperature, and concentrated corrosive chemicals seems to work.
- over 100,000 food cows had mad cow disease but ~170 humans have been diagnosed with it since the British outbreak in the 1990s
- surely more than 170 were exposed–extremely long incubation period, or do we have no fucking clue how prions work?
- given their indestructibility, if the working hypothesis about prion transmission is correct, why is everything not prions?
- (this is comforting, but confusing)