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From Wikipedia [1]: « Prions are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. They character

From Wikipedia [1]: « Prions are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several atal and transmissible neuro degenerative diseases in humans and many other animals. It is not known what causes the normal protein to misfold, but the abnormal three-dimensional structure is suspected of conferring infectious properties, collapsing nearby protein molecules into the same shape. […] There are no effective treatments for prion diseases. » Basically, you get infected, you die. See also [2]. [1]: https://en.wikipedia.org/wiki/Prion https://en.wikipedia.org/wiki/Prion [2]: https://blogs.scientificamerican.com/artful-amoeba/prions-are-forever/ https://blogs.scientificamerican.com/artful-amoeba/prions-ar...

bb123 5y ago
Well we don't really know that, because diagnosis is normally confirmed post-mortem. There could be many people walking around infected with prions that show no symptoms at all.
- deleted 5y ago
  [deleted]
- curryst 5y ago
  There's some pedantry here. There is evidence that some people have a mutation that prevents them from getting CJD. The mutation causes a small change in the proteins that CJD prions impact (changing a glycine to valine at a certain part of the protein). The working theory is that that structural change prevents CJD prions from being able to re-form healthy proteins, preventing the spread of the disease. I wouldn't be at all surprised if those proteins are eventually broken down by the body and removed. All that being said, I don't know if you would even consider it an "infection" if the person is immune to the prions spreading. Prions aren't generally transmissible, so if the prions don't do anything and can't be transmitted... is anything actually wrong? You and I are exposed to tons of bacteria every day, but as long as your body can fight it off (i.e. no symptoms and no transmission), we wouldn't usually call that an "infection". In the same light, presence of abnormal proteins that aren't causing an issue doesn't seem like something we would call an "infection". There's also the fact that some people inherit the disease (as in their body actually makes the prions itself), which doesn't make any sense in the context of an "infection". It's their own body producing the prions, so in those cases it's a genetic disease, not an infection.
rs999gti 5y ago
> Basically, you get infected, you die. Avoid cannibalism and you should be fine. https://www.npr.org/sections/thesalt/2016/09/06/482952588/when-people-ate-people-a-strange-disease-emerged https://www.npr.org/sections/thesalt/2016/09/06/482952588/wh...
- hammock 5y ago
  >Avoid cannibalism and you should be fine. And animal brains, and the areas around experimental research labs...
  - deleted 5y ago
    [deleted]
  - Marsymars 5y ago
    Also see dura mater grafts: https://www.cdc.gov/mmwr/volumes/67/wr/mm6709a3.htm https://www.cdc.gov/mmwr/volumes/67/wr/mm6709a3.htm
- M2Ys4U 5y ago
  Avoid eating British beef in the 1980s/1990s, too. Apparently 1 in 2,000 people in the UK may be infected.[0] We still don't know if there's going to be a second wave of disease from infections from that time period due to genetic factors influencing incubation of the disease. This is why people who were in the UK for longer than a few months during the 80s and 90s aren't allowed to give blood in various places around the world. [0] https://www.bbc.co.uk/news/health-24525584 https://www.bbc.co.uk/news/health-24525584
  - croes 5y ago
    And don't feed beef to cows.
  - yhoneycomb 5y ago
    Serious question should I be avoiding traveling to the UK over this??

bb123 5y ago

Well we don't really know that, because diagnosis is normally confirmed post-mortem. There could be many people walking around infected with prions that show no symptoms at all.

deleted 5y ago
[deleted]
curryst 5y ago
There's some pedantry here. There is evidence that some people have a mutation that prevents them from getting CJD. The mutation causes a small change in the proteins that CJD prions impact (changing a glycine to valine at a certain part of the protein). The working theory is that that structural change prevents CJD prions from being able to re-form healthy proteins, preventing the spread of the disease. I wouldn't be at all surprised if those proteins are eventually broken down by the body and removed. All that being said, I don't know if you would even consider it an "infection" if the person is immune to the prions spreading. Prions aren't generally transmissible, so if the prions don't do anything and can't be transmitted... is anything actually wrong? You and I are exposed to tons of bacteria every day, but as long as your body can fight it off (i.e. no symptoms and no transmission), we wouldn't usually call that an "infection". In the same light, presence of abnormal proteins that aren't causing an issue doesn't seem like something we would call an "infection". There's also the fact that some people inherit the disease (as in their body actually makes the prions itself), which doesn't make any sense in the context of an "infection". It's their own body producing the prions, so in those cases it's a genetic disease, not an infection.

rs999gti 5y ago

> Basically, you get infected, you die. Avoid cannibalism and you should be fine. https://www.npr.org/sections/thesalt/2016/09/06/482952588/when-people-ate-people-a-strange-disease-emerged https://www.npr.org/sections/thesalt/2016/09/06/482952588/wh...

hammock 5y ago
>Avoid cannibalism and you should be fine. And animal brains, and the areas around experimental research labs...
- deleted 5y ago
  [deleted]
- Marsymars 5y ago
  Also see dura mater grafts: https://www.cdc.gov/mmwr/volumes/67/wr/mm6709a3.htm https://www.cdc.gov/mmwr/volumes/67/wr/mm6709a3.htm
M2Ys4U 5y ago
Avoid eating British beef in the 1980s/1990s, too. Apparently 1 in 2,000 people in the UK may be infected.[0] We still don't know if there's going to be a second wave of disease from infections from that time period due to genetic factors influencing incubation of the disease. This is why people who were in the UK for longer than a few months during the 80s and 90s aren't allowed to give blood in various places around the world. [0] https://www.bbc.co.uk/news/health-24525584 https://www.bbc.co.uk/news/health-24525584
- croes 5y ago
  And don't feed beef to cows.
- yhoneycomb 5y ago
  Serious question should I be avoiding traveling to the UK over this??