Prions

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Prions

Prions (/ˈpriːɒnz/), short for proteinaceous infectious particles, are a type of infectious agent composed entirely of protein material. They can multiply by converting related normal host proteins into more prions.

Etymology

The term "prion" was coined by Stanley B. Prusiner in 1982, derived from the words "protein" and "infection".

Description

Prions are unique infectious agents, as they are made entirely of protein. This contrasts with viruses, bacteria, fungi, and other infectious agents, which contain nucleic acids (DNA, RNA). Prions are responsible for a group of diseases known as transmissible spongiform encephalopathies (TSEs), which includes Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy (commonly known as "mad cow disease") in cattle.

Mechanism of Action

Prions propagate by transmitting a misfolded protein state. When a prion enters a healthy organism, it induces existing, properly folded proteins to convert into the disease-associated, prion form; the prion acts as a template to guide the misfolding of more proteins into prion form.

Related Terms

  • Protein Misfolding: A process by which proteins become structurally abnormal and lose their functionality, which is a characteristic of prion diseases.
  • Neurodegenerative Diseases: A group of disorders characterized by the progressive degeneration of the structure and function of the nervous system. Prion diseases are a subset of these disorders.
  • Amyloid: An abnormal protein aggregate that is a hallmark of several diseases, including prion diseases.

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