A prion is a unique type of infectious agent that consists only of a misfolded protein. These abnormal proteins have the ability to trigger the misfolding of other normal proteins, causing a chain reaction within the body. Prions are distinct from viruses, bacteria, and other microorganisms as they lack any genetic material such as DNA or RNA. The name "prion" is derived from the phrase "proteinaceous infectious particle."
Prions are associated with several fatal neurodegenerative diseases, known as prion diseases or transmissible spongiform encephalopathies (TSEs). These diseases affect the central nervous system and lead to severe neurological symptoms including dementia, movement disorders, and a sponge-like appearance of the brain tissue under a microscope.
One of the most well-known prion diseases is Creutzfeldt-Jakob disease (CJD), which can occur spontaneously, be inherited, or acquired through exposure to contaminated tissues. Other prion diseases include variant CJD, Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI).
Prions have a unique ability to resist standard sterilization procedures, such as heat, radiation, and chemical disinfection, making them particularly challenging to eliminate from medical instruments or contaminated materials. They are also known to have long incubation periods, where symptoms may not appear for years or even decades after infection, further complicating diagnosis and prevention efforts.
Given their distinctive characteristics and devastating impact on the nervous system, prions remain an intriguing and significant area of research in the field of infectious diseases and neurology.
