What is prion disease?

Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of rare, fatal neurodegenerative diseases caused by abnormally folded prion proteins.

Prions are misshapen forms of normal proteins found in the body, especially in the brain. These misshapen proteins can trigger normal proteins in the body to become misshapen as well, leading to a chain reaction of protein misfolding and accumulation in the brain.

Prion diseases often have a long incubation period, meaning it can take years before symptoms become apparent. They cause the degeneration of nerve cells in the brain, leading to symptoms such as dementia, ataxia (coordination problems), and changes in behavior and personality. Creutzfeldt-Jakob disease is the most common prion disease, usually affecting older adults.

Prion diseases can be transmitted through infected tissue, such as brain and nerve tissue, as well as through medical procedures that transfer contaminated material. Some prion diseases are hereditary, caused by mutations in the prion protein gene.

Prion diseases are currently incurable and ultimately lead to death.

Source: https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1750-3639.1998.tb00171.x?sid=nlm%3Apubmed

Author: Prof. Dr. Dirk Devroey - Latest update: 2024-09-29 - Copyright: Clinifacts 2024