Prions are infectious neurodegenerative and fatal diseases in humans and animals. Prions are altered type of normal protein known as cellular Prion proteins (PrPc), which are found majorly on the surface of CNS cells. They play an important role in protecting cells and helping cells in responding to oxygen deficiency and metabolism. Prion accumulation in the brain leads to neuronal cell death or degeneration of neuronal tissue.
The misfolding of cellular prion protein (PrP(C)) into pathogenic conformers and protease-resistant isoform PrP(Sc), which leads to a group of fatal neurodegenerative Prion diseases, many diseases like Kuru, Creutzfeldt Jakob disease, and Gerstmann Straussler Scheinker diseases are caused because of them. But on the other side prions are indicated to be beneficial in the process of memory formation.
Keywords: Prion proteins (PrPc), CNS, cellular prion protein (PrP(C)), protease-resistant isoform PrP (Sc).