What are prions, and how do they cause disease? (pages \(138-39\) )

Prions are misfolded proteins that transmit their abnormal shape onto normal proteins, causing several fatal neurodegenerative diseases like Mad Cow Disease and Creutzfeldt-Jakob Disease. Prion diseases, also known as transmissible spongiform encephalopathies (TSE), occur due to the accumulation of misfolded prion proteins in the brain. When misfolded prions contact normal PrP^C proteins, they induce conformational changes leading to a chain reaction of misfolding and the accumulation of toxic aggregates in the brain. This interferes with neuron function, causing cell death and severe neurological dysfunction, eventually leading to death. Prevention mainly involves avoiding contaminated animal products and strict guidelines in medical environments. There are currently no effective treatments, but research is ongoing to find new methods of diagnosis, prevention, and treatment.