What Are Prions?

What are prions?

The word prion was coined in 1982 by neurologist Dr Stanley Prusiner, and is used to describe a proteinaceous infectious agent that is not inactivated or destroyed by procedures that modify nucleic acids such as deoxyribonucleic acid (DNA) or ribonucleicacid (RNA)

Prions differ from other pathogens such as viruses, bacteria, fungi and parasites in that they do not contain deoxyribonucleic acid (DNA) or ribonucleic acid (RNA). They are also non-immunogenic - that is they do not elicit an immune response because the host has been rendered tolerant to the prion by the presence of a normal form of this protein that is produced by the body.

What are prion diseases?

Prion diseases or transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases that affect both humans and animals. These diseasesinclude new variant Creutzfeldt-Jakob disease (vCJD) in humans, bovine spongiformencephalopathy (BSE) or ‘mad cow disease’ in cattle and scrapie in sheep.

What is the origin of prions?

A prion is a modified form of a normal cellular protein known as PrPc, which is foundpredominantly on the surface of neurons. Unlike PrPc, the abnormal protein or prion (PrPSc) is insoluble in detergent and partially resistant to proteinase K.

How are prions transmitted? Prions replicate within the cell by converting normal cell protein into other prions.Ultimately, infected neurons die and release prions that enter, infect, and destroy other neurons. As clusters of cells die, the brain changes and it starts to look spongy. It is thischaracteristic appearance that has led to prion diseases being termed ‘transmissiblespongiform encephalopathies’ (TSE).

Source Pall Medical

What Are Prions?

0 comment(s) for this article

Please leave a comment

Did You Know...?