What common disease is associated with prions?

Creutzfeldt-Jakob disease is a common disease associated with prions, which are misfolded proteins that can induce abnormal folding of normal cellular proteins in the brain, leading to severe neurodegenerative conditions. Unlike traditional infectious agents such as bacteria or viruses, prions lack nucleic acids and are transmitted primarily through contaminated tissues, medical procedures, or inherited genetic mutations.

Creutzfeldt-Jakob disease manifests with rapid cognitive decline, personality changes, and neurologic symptoms, and it ultimately leads to severe dementia and death within a relatively short time frame. The association with prions distinguishes it from other neurodegenerative diseases, which typically have different underlying mechanisms. For example, Alzheimer’s disease and Huntington’s disease are linked to the accumulation of amyloid plaques and mutant huntingtin protein, respectively, while multiple sclerosis involves immune-mediated damage to myelin. The unique pathology of prion diseases, specifically their infectious nature and distinctive protein characteristics, firmly ties Creutzfeldt-Jakob disease to prions.