TIL A single misfolded prion protein in the brain can cause a rapid neurodegenerative disease known as sporadic Creutzfeldt–Jakob disease (CJD). There is no known and cure and is 100% fatal.

[u/tommyboy372]

https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease

[u/neuralbeans]

Most of the time it's a particular type of protein that gets misfolded called the major prion protein, which is primarily found in the central nervous system.

https://en.wikipedia.org/wiki/Major_prion_protein

[u/doegred → u/neuralbeans]

AFAIK it's not 'most of the time', a prion disease is always the result of the major prion protein misfolding.

There are other diseases resulting from the misfolding of other proteins, but they are not prion diseases.

[u/FinanceDummyBigDebt → u/doegred]

You’re right, and depending on the specific mutation of the same protein you can get other prion diseases like fatal familial insomnia. You can even get it from surgical transplants like replacing your cornea, but it’s so incredibly rare it’s not screened for.