What are prions?

Prions are unique infectious agents that are fundamentally composed of protein. Unlike traditional pathogens, such as bacteria and viruses that contain nucleic acids (DNA or RNA), prions lack this genetic material. They are known to cause a group of neurodegenerative diseases by inducing abnormal folding of specific normal cellular proteins in the brain. This misfolding leads to a cascade of protein aggregation, ultimately resulting in neuronal damage and severe neurological symptoms.

Prion diseases, such as Creutzfeldt-Jakob disease, are notable for their insidious onset and progressive fatality. The characteristic feature of prions is their ability to transmit their misfolded shape onto normal proteins, which perpetuates the disease process. This mechanism distinguishes prions from other types of infectious agents, making them a unique category of pathogens.

Understanding prions is essential in the field of infectious diseases because they do not follow the same patterns of replication or response to treatment as traditional microbial infections, presenting significant challenges in diagnosis and management.