Transmissible spongiform encephalopathy

A group of fatal neurodegenerative diseases caused by misfolded prion proteins. They produce sponge-like damage in brain tissue and can spread between susceptible hosts.

Prion: An infectious protein that propagates by inducing a normally folded version of itself to misfold. Misfolded prion protein drives the tissue damage and infectivity characteristic of these diseases.
Creutzfeldt–Jakob disease: A rapidly progressive human prion disease that causes dementia, neurological dysfunction, and death. It is the most common human transmissible spongiform encephalopathy.
Stanley B. Prusiner: An American neurologist who proposed the prion hypothesis and received the 1997 Nobel Prize in Physiology or Medicine. His hypothesis explained how an infectious agent could consist primarily of protein.
Prion disease diagnosis: The clinical and laboratory assessment used to identify suspected prion disease. Diagnosis combines symptoms, brain imaging, biomarkers, and sometimes tissue examination.
Viral infection: A disease process caused by viruses, infectious agents that replicate using host cells and contain genetic material. Prions transmit without the nucleic-acid genome characteristic of viruses.
Prion protein: A host-encoded protein found mainly on the surface of neurons, whose misfolded forms are associated with prion diseases. Its conversion from the normal cellular form to abnormal conformations underlies disease progression.
Variant Creutzfeldt–Jakob disease: A human prion disease linked to exposure to the agent responsible for bovine spongiform encephalopathy. It demonstrates that an animal prion disease can cross into humans.
Prion hypothesis: The proposal that prions transmit disease through protein conformational change without requiring a nucleic acid genome. It accounts for the unusual agent responsible for these encephalopathies.
Real-time quaking-induced conversion: A laboratory assay that detects prion seeding activity by amplifying protein aggregation in vitro. It can detect prion activity in cerebrospinal fluid or other specimens during diagnostic evaluation.
Bacterial infection: A disease process caused by bacteria, single-celled organisms capable of independent cellular functions. Prions are infectious proteins rather than living microorganisms.

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Daniel Carleton GajdusekNarrower topic: Kuru became a landmark example in this family of diseases.