#gujaruto-gujhe-gujear

[prionguy-old]

Hello I came across one of your reblogs and yes I'd like to learn more about prion diseases

HI

yes this has been sitting in my inbox for hours we dont talk about that

okok ! so basically, prions are naturally occuring proteins in the brain (the function of these is actually unknown!). however, when they misfold, that misfolding copies to other prions, which causes prion diseases.

(heres what i mean by misfolding)

and these diseases can be found in a decent variety of animals? including sheep, goats, cows, deer, cats, camels, and of course humans.

for sheep and goats, its scrapie, which causes excessive itching and weird mouth movements, and heavy weight loss

for cows, weve got the most well known, mad cow disease. it causes aggression and bad coordination (this can spread to humans actually, causing creutzfeld jakob disease)

in deer, its chronic wasting disease ! hoo boy this was the thing talked about in kaijuno’s post, its WILD

cats and camels have specific variants of those that i dont know much about

AND IN HUMANS. prions cause 6 known diseases (cjd, kuru, fatal familial insomnia, gss, fse, VSPSr), but i only know a lot about the first three.

creutzfeld jakob disease is the most common as far as i know. it causes very rapidly progressing dementia, and VERY quick death (usually around a year for genetic cases, but can be as little as two weeks from the first symptoms for sporadic cases). 87% of cases are of unknown cause which is WILD

kuru. this disease is (as far as we know) extinct. it was very present among the fore tribe of papua new guinea in the 1900s. its likely that a member of the tribe got a form of cjd, and it mutated. the way that it spread so fast was actually because they participated in uh. funerary cannibalism. needless to say they do not do that anymore. but this disease was wild, with about a year life expectancy from the first symptoms but around a 50 YEAR long incubation period. symptoms included bad coordination, weight loss, and chillingly, random laughter.

finally, fatal familial insomnia, my personal favorite. i recommend the book “the family that couldn’t sleep” on this because its great :0. its almost exclusively genetic, and (obviously) a symptom is trouble sleeping. the patient fully loses their ability to sleep and subsequently. dies. since it is genetic, there is are families worldwide who pretty much all have a 50/50 chance of developing this disease.

the only things these all have in common is that theyre caused by malformed prion proteins, they are EXTREMELY rare, and they are always fatal. theyre also super interesting because as humans, we have always known diseases as Living Things like bacteria or viruses. however, proteins arent living. you cure viruses and such by killing the viruses, but well, how do you kill a protein?

if you wanna learn more about this, i just recommend looking up the diseases!! i also recommend these books

- madness and memory by stanley prusiner (the guy who discovered prions)

- the family that couldnt sleep by dt max

- mercies in disguise by gina kolata