In humans, prions are believed to be the cause of Creutzfeldt–Jakob disease (CJD), its variant (vCJD), Gerstmann–Sträussler–Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru.
What diseases are caused by prions?
Identified Prion Diseases
- Creutzfeldt-Jakob Disease (CJD)
- Variant Creutzfeldt-Jakob Disease (vCJD)
- Gerstmann-Straussler-Scheinker Syndrome.
- Fatal Familial Insomnia.
What are prions?
A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. Prion diseases can affect both humans and animals and are sometimes spread to humans by infected meat products. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD).
What can kill prions?
To destroy a prion it must be denatured to the point that it can no longer cause normal proteins to misfold. Sustained heat for several hours at extremely high temperatures (900°F and above) will reliably destroy a prion.
What is true about prions?
The misfolded proteins known as prions are very real. Prions are proteinaceous infectious particles, formed when normal proteins misfold and clump together. But a different side of prions is also coming to light. Many newly discovered prions and prion-like proteins do not appear to cause disease at all.