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Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

blogs.scientificamerican.com

71–80 of 195 posts

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#71

Earlier quoted context omitted.

It's actually relatively simple. Proteins, very roughly speaking, are things that change other things into something we need. You could think of proteins required for breaking down sugar, which do more or less exactly that: they take a molecule of sugar, break a part off of it and hand it to the next protein in the chain. What makes prions so weird is that they are a protein that has been mismade (misfolded, generall…

>What makes prions so weird is that they are a protein that has been mismade (misfolded, generally). And science doesn't know how they get misfolded in the first place then they basically go "hey other protein, boop you're like me now, let's burn this mother to the ground" and then you get something similar to the 'corrupted blood incident' https://en.wikipedia.org/wiki/Corrupted_Blood_incident except you lose your m…

Proteins fold based on minimizing energy states. Any amino acid sequence (large molecules) can be folded in many different ways. For example enzymes binding to proteins can cause desired protein folding changes. Generally if a protein gets misfolded (happens all the time) it doesnt do anything, in some cases misfolding causes bad effects.

Prions misfold in a way that induces other of the normal version to misfold in the same way and therefore spread.

On the plus side prions kills pretty quickly and so there is unlikely to be much underlying mutation in their form or function.

Protein folding is incredibly complicated but I could imagine enzymes which could snip the prion or another similar protein which induces it to fold back into the preferred state. I doubt we have the science to explicitly design such a thing, but you could expose prion molecules in bacteria and put them under evolutionary pressure and they might eventually develop something that disables/fixes the prion.

You would essentially align survival of the bacteria to disruption of the prion molecule.

This link has an image of the two versions

https://www.mayoclinic.org/diseases-conditions/creutzfeldt-j...

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#72
post #61

Earlier quoted context omitted.

I believe Alzheimers is recognized as a metabolic disease now (some have even said Type 3 diabetes). It's in no way related to Prions.

The diabetes/metabolic theory was one pitched theory, and it isn’t even a terribly mainstream one. There is no consensus that Alzheimer’s is a “metabolic disease”. Even thought ApoE is implicated, that association could be explained by numerous mechanism, even viral.

Thanks for clarifying. I'm no expert just passing on things I've read.

Regardless, it's unrelated to prions.

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#73
post #63
post #7

About 20 or so years ago I was feeling bad about my chances of developing the type of CJD associated with mad cow disease because of all the burgers I'd eaten over the preceding years. There were also human cases getting reported at a rate that some suggested matched the pattern for the early days of an epidemic. Luckily that never materialised. Still though having lived in the UK during a certain time period preclud…

There were certain feeding practices that were banned or reduced that were thought to contribute. I.e. many places quit feeding meat and bone meal of other livestock to cattle. https://en.wikipedia.org/wiki/Cattle_feeding#Mad_Cow_Disease

That surely helped but as always, some enterprising folks found a way to "launder" the goods by feeding the banned products to poultry and then feeding their remnants and droppings to cattle.

Compare US position: https://www.feednavigator.com/Article/2017/07/22/USDA-Feed-n...

VIC position: http://agriculture.vic.gov.au/agriculture/livestock/beef/fee...

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#74
post #26

Given that prions are proteins, why can't we use immunotherapy and vaccinate?

Well the immune system needs to be able to do something of use in response. Just putting it in even with an immune sensitizing agent would add another prion that it can't do anything useful about. But antibodies, if we had ones that worked for a strain and weren't essentially prions themselves we could vaccinate for it or use it to "chelate" assuming the prion grabbers wouldn't cause other unacceptable problems.

Thanks for replying (some people have downvoted my question, which is weird).

I don't completely understand your answer, due to my ignorance of the immune system. I thought the immune system could target specific proteins (e.g. how snake venom vaccines work) rather than using the proteins to identify target cells. Is this correct? I as assuming such a mechanism would be able to do something useful with the target.

The other part of your answer I did understand, and that was helpful, thanks!

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#75
post #39

I had project in college on Prions. Some interesting things I remember: * Kuru (laughing disease) was transmitted from cannibalism. Women and children of the tribe were more likely to contract it because they were more often given the less palatable parts of the bodies, which included brains * Some countries do not trust American cattle because our standards for Prion screening are too low * Kentucky has issues with…

Cooked goat and cow heads are a delicacy in some regions of Tunisia. Obviously, this includes the brain. I now wonder if Prions have ever been an issue in Tunisia...

Goat heads in Iceland too.

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#76
post #26

Given that prions are proteins, why can't we use immunotherapy and vaccinate?

Well the immune system needs to be able to do something of use in response. Just putting it in even with an immune sensitizing agent would add another prion that it can't do anything useful about. But antibodies, if we had ones that worked for a strain and weren't essentially prions themselves we could vaccinate for it or use it to "chelate" assuming the prion grabbers wouldn't cause other unacceptable problems.

Passive immunization might indeed be a feasible option, but of course requires timely detection.

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#77

I had project in college on Prions. Some interesting things I remember: * Kuru (laughing disease) was transmitted from cannibalism. Women and children of the tribe were more likely to contract it because they were more often given the less palatable parts of the bodies, which included brains * Some countries do not trust American cattle because our standards for Prion screening are too low * Kentucky has issues with…

> Try not to eat animal or human brains

Now all I can think about is the dinner scene at the end of Hannibal.

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#78
post #74

Earlier quoted context omitted.

Well the immune system needs to be able to do something of use in response. Just putting it in even with an immune sensitizing agent would add another prion that it can't do anything useful about. But antibodies, if we had ones that worked for a strain and weren't essentially prions themselves we could vaccinate for it or use it to "chelate" assuming the prion grabbers wouldn't cause other unacceptable problems.

Thanks for replying (some people have downvoted my question, which is weird). I don't completely understand your answer, due to my ignorance of the immune system. I thought the immune system could target specific proteins (e.g. how snake venom vaccines work) rather than using the proteins to identify target cells. Is this correct? I as assuming such a mechanism would be able to do something useful with the target. Th…

It's definetely being looked at, for example: https://www.ncbi.nlm.nih.gov/pubmed/18215090/ (paywalled).

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#79
post #54
post #49

Earlier quoted context omitted.

Just when I thought my home state couldn't embarrass me more by being backwards... Thanks!

I wouldn't be embarrassed. Those squirrels were probably wild caught (vs factory farmed), and the fact that they didn't waste the brain shows their resourcefulness. Those are,after all, the same things we (rightfully) laud in the consumption of other types of more normative food animals. Just because it's a squirrel shouldn't change that. EDIT: Added editorial wording to second paragraph.

Absolutely. Though as someone who has eaten squirrel (WV native. Yes it tastes like chicken.), I am amazed that someone took the time to get the brains out and eat them. It takes several squirrels to make a decent meal. I can't imagine how many it would take to have a substantial amount of what must be very small brains.

Re: Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims

#80

Earlier quoted context omitted.

Infectious disease is one area where fear of the unknown is rational. We don't just need to defend against existing diseases, but also diseases yet to emerge. Just because CJD is rare doesn't mean that the next prion disease will be, so it would make sense to take measures to prevent transmission of prions now.

I don't think that's true. Prions aren't bacteria or viruses that evolve and transmit. They're mostly just a misfolded protein that occurs spontaneously. (They can then be transmitted.) I'm not sure there will ever be a prion that hasn't already existed, whereas I am sure there will be new viruses and bacteria.

If they can replicate imperfectly, they're subject that evolutionary processes.
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