• Dolores [love/loves]
    ·
    1 year ago

    so what i don't understand is how prions can be resisted, and whether the epidemiology of a prion disease is about who ingests/receives an incorrectly folded protein, or about individuals with particularly... foldable? proteins getting a prion and developing an illness that others wouldn't. i'm particularly confused because the immune system isn't involved, is it?

    • Clever_Clover [she/her]
      ·
      1 year ago

      so what i don't understand is how prions can be resisted

      basically all prion diseases that we know of are not curable and have a 100% fatality rate

      and whether the epidemiology of a prion disease is about who ingests/receives an incorrectly folded protein

      well, it can depend on some things, like for example if there was a difference in how well your body worked without that certain protein being functional compared to another person who is less resilient when this particular protein doesn't work well, it's kind of the reason why the incubation period varies so widely, the build up and conversion from good proteins to misfolded ones isn't instant and takes time, so until that build up gets to a level where it causes symptoms we just don't know that the person has anything wrong with them

      or about individuals with particularly... foldable? proteins getting a prion and developing an illness that others wouldn't.

      there's variation between people of course, but no if a prion is a certain protein and you expose it to proteins of the same kind it makes them misfold, maybe certain people have a different variation of that protein that does the same job but is structured a little differently so it's not as susceptible or at all even, but that's speculation on my part, I haven't read deeply into this particular topic, generally speaking research into prion disease is still very inadequate, it's very rare, and is always fatal, so it's kind of hard to do research on

      i'm particularly confused because the immune system isn't involved, is it?

      yeah the immune system can't really fight it typically, because the protein matches the ones that the body uses, there isn't much to tip it off since the immune system doesn't look at protein folding, it looks at what proteins, sugars, and other molecules something has, in fact, the immune system can actually help the prion disease move into the body and reduce the incubation period as was seen in this study on rats, https://sci-hub.se/https://doi.org/10.1006/clim.2000.4875

      oh also, not every exposure would mean 100% getting infected, especially since the propagation of the misfolding isn't instant and takes time, so if those proteins get disposed of and replaced before they can spread (as the body naturally does dismantle and rebuild lots of proteins) then you wouldn't get infected

      though keep in mind that these are rare diseases, that affect like 1-2 people per million worldwide every year, some of those cases are due to infection (acquired), some are due to genetic issues that are inherited, meaning the body itself makes misfolded proteins sometimes due to genes being coded with a bad mutation in the area that codes for that protein, and some are just sporadic, meaning one protein just decides to misfold because it got built slightly wrong cause that person is extremely unlucky and then it spreads

      more reading here https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4890484/

      generally speaking, not something you wanna ever risk the chance of making more common

      • Dolores [love/loves]
        ·
        1 year ago

        hell yeah i really appreciate you taking the time :mashallah:, thank you

        i'm fascinated to know how that chance of being infected stacks up against life expectancy, but that's gotta be an insane thing to study---probably a majority of possible prion-disease uhhh 'infected' might expire from other things before a decade or two of incubation actually completes. its not like we're doing detailed studies of every dead person to check.

        extremely interesting, the only subject that's prompted me to consider going into biology