Bioinformatics Education Dissemination: Reaching Out, Connecting and Knitting-together

Background

Stanley Prusiner first proposed the term “prion” in the early 1980s. He found evidence of neurological diseases caused by agents that appeared resistant to the processes that normally destroy nucleic acids. Once highly controversial, the idea won Prusiner the Nobel Prize in Physiology or Medicine in 1997.

Diseases caused by prions are characterized as spongiform ncephalopathies. Holes develop throughout the brain. Cells begin to die from the abnormal prions’ ability to link together and form long protein rods. The tissue damage causes progressive degeneration in neurological control that can affect things like balance, muscular control, mood, and sleep (depending upon the area of the brain most affected). All prion-caused diseases are fatal.

Prions have both genetic and infectious etiologies. All mammal cells carry a gene that produces the normal form of the protein, PrPC. Sporadic or inherited point mutations in this gene can cause disease. But prions can also cause diseases by passing between individuals. The abnormal form can induce conformational change in the normal prions already present in the neurological tissues, producing more abnormal forms. These forms can then cause further conformational changes in a positive feedback loop.

The function of PrPC is not completely understood. It is concentrated in the cellular membranes of neurons and may play a role in synaptic communication. Mice missing the prion gene appeared to develop no ill effects, but also failed to develop symptoms of the disease when exposed to prions.

The general public became interested in prions during the 1990s when Great Britain discovered an epidemic of bovine spongiform encephalopathy, or Mad Cow Disease. The discovery that people might be affected by prions ingested from beef made headlines around the world. The United States announced the first case inside its borders in late 2003. Midwestern deer and elk populations are affected by chronic wasting disease, a prion disease of concern to hunters and game animal farmers.

 

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