Which human diseases are caused by prions?

Prions are abnormal, misfolded proteins that accumulate in the brain, causing fatal neurodegenerative conditions known as transmissible spongiform encephalopathies. These agents are unique because they lack nucleic acids and are highly resistant to enzymatic degradation, leading to the destruction of neurons and the formation of sponge-like holes in brain tissue.12

Human diseases caused by prions include:

• Creutzfeldt-Jakob disease: A rare, progressive, and fatal condition characterized by dementia and loss of muscular coordination. It can occur sporadically, through inheritance, or via infection.13
• Gerstmann-Sträussler-Scheinker disease: A rare, inherited prion disorder.1
• Fatal familial insomnia: A progressive disease that severely impacts sleep and autonomic functions.1
• Kuru: A degenerative disorder of the central nervous system that was historically transmitted through ritualistic cannibalism in Papua New Guinea.12

While these conditions are distinct, they share a common pathology involving the conversion of normal brain proteins into the pathogenic prion form.12