Anti-Prion Protein Antibody

Cell Markers, Neuronal Markers

United States

27 kDa

Overnight Priority

Prion protein is the protein responsible for human Creutzfeldt-Jakob Disease (CJD), and somatic mutations of the prion gene are responsible for Gerstmann-Straussler-Scheinker disease and Fatal Familial Insomnia. The bovine homolog of the prion protein is responsible for bovine Spongioform Encephalopathy (BSE), also known as “, Mad Cow Disease.”, In all cases, the prion protein has two folding states, with one state being the “, normal”, non-pathological form, and the other state inducing a spongioform encephalopathy.

Antibodies were prepared by injecting laying hens with purified recombinant human PRN protein. After a series of boosts, eggs were collected from hyperimmunized aniamsl and the IgY fraction prepared. Affinity purified antibodies were then isolated from the IgY fraction using a PRN protein column. Finally, affinity purified antibodies were mixed with the IgY fraction to make the final product. These antibodies have been validated with human, mouse and rat tissues.

Prion protein is a ubiquitously-expressed, 27661 dalton protein (253 amino acids, NP_898902.1) of unclear function(s) in eukaryotes. This protein can exist in one of two conformational forms. One is its normally-folded form (PrPc or "cellular PrP"), which contains a mixture of alpha helical regions and a few beta-pleated sheets. The other is a misfolded form (PrPsc or "scrapie PrP") whose structure contains mostly beta-pleated sheets. This misfolded scrapie PrP form is cytotoxic, especially to nervous tissue, and can serve as a "seed, " inducing adjacent, normally-folded PrP proteins to misfold into the cytotoxic conformation. In this sense, the scrapie PrP can be thought of as an infectious agent both within the organism as well between organisms.

Polyclonal

1:1000-1:2000

Recombinant fragment of human prion

Mixture of IgY fraction and affinity-purified antibodies

These antibodies are to be used as research laboratory reagents and are not for use as diagnostic or therapeutic reagents in humans.