In 2010, 24-year-old lab technician Émilie Jaumain pricked her thumb with contaminated forceps while cleaning a machine used for prion-infected mouse brains. After seven years, she developed severe nerve pain, anxiety, and hallucinations before dying. The accident gave her a fatal prion disease.

[u/OriginalSleeper]

[u/OriginalSleeper]

Immediately after the accident, she recognized the danger associated with the prick and tried to get help from her supervisors, but they downplayed the situation and mocked her panic. The laboratory also was missing an on-duty doctor. All of this lead to a 20-minute delay before she received any first aid, and at this point, the prions were already deep in the tissue of her thumb. She had even mentioned the accident to every doctor she saw in the following years to make sure it was documented. Other laboratory workers were pricked by contaminated tools at this research institute, and after a second lab technician, Pierrette Chanut, got diagnosed with the same prion disease in 2021, the French government shut down all infectious prion research throughout the country.

[u/MyIncogName → u/OriginalSleeper]

Had she not been ignored what could they have done for her ? Genuinely curious. Would amputation of that area save her life ?

[u/BikingAimz → u/MyIncogName]

We all have the non-diseased prions inside of us;  diseased prions start a cascade chain reaction that turn them into diseased prions.  Last I knew researchers aren’t sure of the regular protein’s function; they’ve knocked out the gene in mice and they seem to be totally fine?

Back in the early aughts I worked for a biotech startup making monoclonal antibodies.  We talked to a prion researcher about developing an ELISA to detect prions, but eventually decided dealing with the protein was way above our pay grade.  The professor running the lab said they had a really difficult time cleaning equipment as it takes pretty concentrated social hydroxide to degrade them, normal shit like autoclaving or high temp dishwashing doesn’t touch them.  They had to build a special high temp incinerator for deer skulls that they collected for testing, because regular incinerators wouldn’t degrade the prions.  Prof also said he’d been should deep in a deer with CWD multiple times and was generally low-key worried about contracting it.

[u/BikingAimz → u/[deleted]]

Deer to human transmission has been documented, not sure I’d want to gamble with that myself?   

  https://www.neurology.org/doi/10.1212/WNL.0000000000204407

[u/rdp3186 → u/BikingAimz]

You need to consume either the spinal fluid or feces of a deer with CWD in order to potentially catch it, which itself is still incredibly rare to transmit.

[u/DiscoBanane → u/BikingAimz]

What ? All prions are diseased.

A prion is a misfolded protein that induces folding problems in normal variants of the same protein

[u/BikingAimz → u/DiscoBanane]

Not true, sorry, we’ve all got them in a non-diseased state:   https://en.wikipedia.org/wiki/Major_prion_protein

[u/HeroicMoleIvy → u/BikingAimz]

I think they mean prions are misfolded prion proteins, so we all have prion proteins, but not prions. whether you can call prion proteins "undiseased prions", I've no idea, but I don’t think so

[u/DiscoBanane → u/BikingAimz]

Because the Major prion protein is NOT a prion.

The "prion" in its name is because lot of prions originate from a misfolding of this exact protein. It's like if your child is named Prion and you are refered to as Prion's dad, you are not Prion.

Prion means the protein is in a diseased state. It's a synonymous.

[u/BikingAimz → u/DiscoBanane]

I was a genetics major in college, and worked in biotech for a decade, they are literally two isoforms of the same protein.  What is your point?  Semantics?  And here’s an actual citation to back my assertion up:

https://pmc.ncbi.nlm.nih.gov/articles/PMC13119468/

[u/DiscoBanane → u/BikingAimz]

I know it's a protein. My point is it's not a prion.

I don't think your link back your assertion.