Prion Diseases

Human Prion Diseases Easily Explained

Prion Definition

1. What is a prion?

A prion is an infectious (transmissible) protein able to replicate by transforming other proteins into a copy of the prion. The mechanism of copying is not yet understood by science.

The hypothesis was developed based on research on a nervous system disease known as Creutzfeldt-Jacob disease, which is epidemiologically associated with a bovine disease called bovine spongiform encephalitis (the mad cow disease). Research discovered that the infectious agent that causes those diseases, surprisingly, was a protein capable of copying itself and of being transmitted by ingestion (the reason why meat from contaminated animals cannot be consumed), inoculation and even heredity.

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Prion Diseases - Biology Questions and Answers

Human Prionic Diseases

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2. What are the main human diseases caused by prions?

The main known human diseases caused by prions are Creutzfeldt-Jacob disease (CJD), kuru and Gerstmann-Sträussle-Scheinker disease (GSS). The hypothesis that many other diseases of unknown etiological agents are actually caused by self-replicating infectious proteins is strong.

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