Proteins are extremely important for our everyday life, but what happens when these proteins decide to become silent killers? Some proteins, even though really rare, can misfold from prion, which actually misfold other properly folded proteins causing an exponential chain reaction of misfolded proteins that all end up binding together. In an infected person, these proteins are located in the brain and cause neural issues and death quite quickly. These neurological disorders are known as transmissible spongiform encephalopathies (TSEs). They are a group of the rarest diseases and have the highest mortality rate on the planet. There are no known cures or treatments available and these proteins can’t be denatured by proteases, enzymes, heat, radiation, and even autoclaves, which are meant to kill any and all bacteria and viruses.
So how do you get this disease? It’s quite complicated and of course more research needs to go into it, but there are several ways we think we can get it. It starts off by knowing there's a cow version of it called Mad Cow Disease, which you’ve probably heard of. If humans eat beef infected with prions and the prions make it to the brain, it can induce the disease. It's also thought that if humans come in contact with manure or urine from an infected cow that there is a chance of being infected as well. Additionally, wows get it the same way humans do, by eating infected meat. The good news is that the U.S. banned the practice of feeding cattle other cow parts in 1997 to minimize the risk of reinfection. Kuru is another version in which human cannibalism can cause it. People found with Kuru end up with it from eating human brains that are already infected, a common practice in funerary rituals in New Guinea. There’s also an even rarer genetic version, Creutzfeldt-Jakob disease, which causes progressively worsening insomnia. This then leads to delirium and a confused state of dementia where you get so sleepy that you go crazy and eventually die.
Even though prions and the diseases they cause are extremely scary, we have a lot of preventative measures to keep them hopefully out of the population. More research needs to be done on the prions to understand ways to combat them and possible treatments for the issues they cause. Luckily, TSEs are extremely rare and only affect about 1 in every million people each year, so these symptoms are rarely suffered. If we can find a way to disarm prions we can make sure that these proteins don’t decide to go rogue.
https://pubmed.ncbi.nlm.nih.gov/9637955/
Timinte Abraham (7)
I've heard of some of these diseases before but I didn't know about Kuru. Do the people who practice those rituals know the danger behind cannibalism? Also for Creutzfeldt-Jakob disease, if it is genetic is there a certain age were one would notice they have it?
ReplyDeleteI had never heard of Kuru before doing more research for this blog post either, but I was familiar with Mad Cow and Creutzfeldt-Jakob disease. I think people with sporadic CJD are able to identify symptoms in their 60s, but with variant CJD people can identify them in their 20s.
DeleteI've heard about mad cow disease and that it can affect us, but I didn't know it was to that extent. It's really crazy to think about and thankfully it is extremely rare to obtain, but it's still obtainable. I agree that there should be more research and studies on it so we can eventually treat the disease and help those who do contract it. Very informative post!
ReplyDeleteI think the fact that there is so much more to find out about prions and what can ultimately destroy them drew me to this topic post, but I'm glad you found it as interesting as I did!
DeleteHello,
ReplyDeleteI have heard about Kuru before and it was interesting to read more in depth about it. It is interesting how the people who are infected do not influence others who have similar diets. I wonder if they do anything to prevent it or they are willing to continue despite the possible consequences.
- Tugba Kahveci
It would be smart to have preparation methods in place to reduce the risk of being infected. I'm thinking that there are no current prevention methods because there also aren't any known ways to denature prions, but definitely a question to consider.
DeleteHello,
ReplyDeleteI've heard of mad cow disease but never knew that the disease was able to affect humans in such a way.
-David Miropolsky
Yeah it seemed interesting to learn more about when I was trying to choose a topic.
DeleteI've only heard about mad cow disease but not Kuru. I wonder if these diseases will become more common as time goes on and as more and more diseases arise.
ReplyDelete- Hannah K
This is why it is very important to properly cook meat, I didnt realize how dangerous this actually was.
ReplyDelete-Lara Pereira