Pathogenicity and Effects of Prions Misfolding

Abstract

Prions are proteins found naturally in the human body and also in other species. Prions are ubiquitous and have been found in everything from plant and mammal cells to single celled organisms such as the bacterium, Escerichia coli. The natural function of prions is largely unknown, however it is thought that they are involved in several functions such as cellular adhesion, protection, and cell signaling. The pathogenicity of prions stems from an altered pattern of folding of the protein.The altering event that triggers change is a single amino acid replacement which converts one of the alpha helices in the cellular prion protein into a beta sheet confirmation. This specific mutation induces the pathogenic isoform This isoform can interact with the normal celular versions of prion to convert them into the pathenogenic forms. This paper will look at prion diseases and how they affect the brain as well as what contributes to their pathogenicity and transmission.