Jeonbuk Nat'l University researchers trace brain’s fatal decay to ‘iron death’
Researchers in Korea have uncovered a key mechanism behind prion diseases, rare brain disorders caused by misfolded proteins that destroy nerve cells and are almost always fatal. The study, led by Jeong Byung-hoon at Jeonbuk National University, found that ferroptosis — a form of iron-dependent cell death — is a key driver of nerve cell destruction in prion diseases. Prion diseases are deadly neurodegenerative disorders caused by the buildup of misfolded proteins in the brain. They include Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy, or mad cow disease, in animals. Despite their severity and rapid progression, no effective treatments currently exist. The research team conducted an integrated analysis using brain tissue from patients with sporadic Creutzfeldt-Jakob disease, as well as infected mouse models and cultured human cells. The findings showed a significant decrease in the antioxidant enzyme GPX4 and an increase in lipid peroxidation markers, both hallmarks of ferroptosis. At the cellular level, prion peptides caused cells to produce more reactive
