Prion Protein’s Tail Responsible for Brain Cell Death

Infectious pathogens known as prions are responsible for illnesses like Mad Cow Disease. But recent news points to the true cause of the protein’s destructive nature, and why some prion proteins remain completely harmless while others become toxic. Prion protein occurs naturally in the human brain cell membrane. Sometimes, those normally harmless proteins will morph or become deformed. It’s these abnormal and deformed versions of prion proteins that become deadly to brain cells. But the real discovery came when researchers were able to demonstrate the differences between the two main components of the prion protein molecule. As it turns out, the prion protein is comprised of both a globular domain and an unstructured, long tail. It’s this tail, normally responsible for assisting in nerve cell functions, that can lead to cell death. It appears that during a pathogenic infection, the prion protein’s tail deforms and interacts with the globular section, which is what leads to the death of brain cells. The scientific discovery of the tail deformity within the protein leading to cell death is a big leap towards understanding, treating, and eventually eradicating prion diseases and disorders.