PRIONS

Prions are proteins found on the plasma membrane (the membrane that surrounds a cell and defines its physical boundary). In mammals, prions are found in the highest concentration in cells of the central nervous system. In mammals and yeast, there are several genes coding for different prions.

The function of normal prions (denoted PrPC) is unknown. Aberrant or mutant prions (denoted PrPSc) are thought to be the causative agents of a set of neurological disorders, among which are BSE in cows and Creuzfeldt-Jacob Disease (CJD) in humans. The term prion comes from "proteinaceous infectious particles". The term was coined during early research of the sheep disease called scrapie. During that time, all that was known about the particle that caused scrapie was that it was a protein and that it was infectious.

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Prion protein is expressed on long-term repopulating hematopoietic stem cells and is important for their self-renewal.