Prion

Most people haven't heard of the word prion however they have heard of mad cow disease. This disease along with Creutzfeldt-Jakob disease (CJD) in humans is caused by a prion, an infectious particle made mostly of protein. A prion is a misfolded form of the PrP protein. Prions do not replicate, they cause other PrP proteins to become misfolded. Prions cause proteins to form into an amyloid, or a tightly packed beta sheet. These packed beta sheets are extremely dense and very stable. When they accumulate they become extremely resistant to denaturation and chemical treatment. Prions accumulate extracellularly within the central nervous system. Diseases caused by prions are untreatable and fatal. Researchers are scrambling to find answers to the unanswered questions about diseases caused by prions. EMD, Abgent, and G-Biosciences provide products used in prion-based research.