Prions, abbreviated from “proteinaceous infectious particles”, are misfolded proteins capable of transmitting their abnormal shape to normal cellular proteins, triggering fatal neurodegenerative diseases in humans and animals. Unlike bacteria or viruses, prions lack genetic material (DNA or RNA) and propagate solely through structural corruption of the major prion protein (PrP). Normally, PrP exists as a harmless, alpha-helix-rich protein (PrPC) on cell surfaces. When misfolded into a beta-sheet-dominant form (PrPSc), it aggregates into toxic clumps that destroy brain tissue, creating microscopic holes that give the brain a sponge-like appearance.

Mechanisms of Pathogenicity

The danger of prions lies in their unique properties:

Documented Human Prion Diseases

Major forms include:

Why

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