abnormal prion protein

Abnormal prion protein is a misfolded form of a naturally occurring protein in the brain. While normal prion proteins help maintain cell function, the abnormal form is shaped differently and can cause other normal proteins to also misfold. These misfolded proteins aggregate and form deposits that damage brain tissue, leading to neurodegenerative diseases known as prion diseases (e.g., Creutzfeldt-Jakob disease). The abnormal prion protein is resistant to usual degradation processes, making it difficult for the body to eliminate, and its presence disrupts normal brain function, resulting in rapid cognitive and motor decline.