The most difficult prions riddles

All known prion diseases affect the structure of the brain or other neural tissue and all are currently untreatable and universally fatal. This includes bovine spongiform encephalopathy (BSE, also known as "mad cow disease") in cattle and Creutzfeldt–Jakob disease (CJD) in humans.

The smaller the agent, the more difficult it is to destroy it. Prions are quite resistant to proteases, heat, radiation, and formalin treatments. 134°C (274°F) for 18 minutes in a pressurized steam autoclave may not be enough to deactivate the agent of disease. Prions may last for decades in the environment.

A prion is a single protein in a misfolded form. The prion theory was developed to explain the discovery that the mysterious infectious agent causing the diseases scrapie and Creutzfeldt–Jakob disease resisted ionizing radiation. The data suggested that the infectious agent was too small to be a virus.

A prion is composed of protein in a misfolded form. When a prion enters a healthy organism, it induces existing, properly folded proteins to convert into the disease-associated, prion form; the prion acts as a template to guide the misfolding of more proteins into prion form.