testing for prions, especially for ones that you don't know of yet (which can develop at any time in any person due to the nature of prions, but that's very very rare) is extremely difficult, when mad cow disease is detected in a cattle herd you cull the herd, it isn't worth it, the risk is far too high, and prions cannot be removed through anti-biotics or any kind of treatment, the only way to destroy them is denaturation through burning
prions are misshapen proteins that 'spread' in that when they touch the normally shaped proteins of the same kind they can cause them to become misshapen in the same way too, and being misshapen means this protein can't do its job, and when it's job is keeping your brain working or doing other important biological functions, you can see where issues arise
someone who was healthy at the end of their life may have prions in their body that were dormant and did not show symptoms, the longer you live the more likely you are to have proteins become misshapen, most are harmless and wouldn't spread anyway, and most people die without developing anything to do with prions because of just how unlikely it is to happen
but in the rare chance someone's body produces a misfolded protein that is both infectious and affects an important biological function then that extremely small chance would be buried in the ground or incinerated the majority of the time, but if more people practiced cannibalism then that protein would have a field day as it spread to the first person and then through blood contact spread to others, because a lot of prion disease is slow acting and/or has a very long incubation period (multiple years)
so not practicing cannibalism makes it a lot less likely for an untreatable deadly prion disease to materialize
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?
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
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
testing for prions, especially for ones that you don't know of yet (which can develop at any time in any person due to the nature of prions, but that's very very rare) is extremely difficult, when mad cow disease is detected in a cattle herd you cull the herd, it isn't worth it, the risk is far too high, and prions cannot be removed through anti-biotics or any kind of treatment, the only way to destroy them is denaturation through burning
prions are misshapen proteins that 'spread' in that when they touch the normally shaped proteins of the same kind they can cause them to become misshapen in the same way too, and being misshapen means this protein can't do its job, and when it's job is keeping your brain working or doing other important biological functions, you can see where issues arise
someone who was healthy at the end of their life may have prions in their body that were dormant and did not show symptoms, the longer you live the more likely you are to have proteins become misshapen, most are harmless and wouldn't spread anyway, and most people die without developing anything to do with prions because of just how unlikely it is to happen
but in the rare chance someone's body produces a misfolded protein that is both infectious and affects an important biological function then that extremely small chance would be buried in the ground or incinerated the majority of the time, but if more people practiced cannibalism then that protein would have a field day as it spread to the first person and then through blood contact spread to others, because a lot of prion disease is slow acting and/or has a very long incubation period (multiple years)
so not practicing cannibalism makes it a lot less likely for an untreatable deadly prion disease to materialize
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?
basically all prion diseases that we know of are not curable and have a 100% fatality rate
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
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
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
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