Nucleic acid encoding prion protein variant

Abstract

A protein designated Prion Protein Modulator Factor (PPMF) is disclosed which protein is an auxiliary factor in prion replication. PPMF is primarily characterized by its ability to bind to PrP.sup.C and facilitate a conformational change from PrP.sup.C to PrP.sup.Sc. A discontinuous epitope on PrP.sup.C comprising residues 172, 215 and 219 of human PrP.sup.C binds PPMF which is encoded by a nucleotide sequence derived from an organism selected from the group consisting of cow, sheep, mouse, hamster and human. In converting PrP.sup.C to PrP.sup.Sc the PPMF forms a PrP.sup.C /PrP.sup.Sc complex and is a rate limiting compound in the formation of that complex. Molecules, including antibodies, which bind PPMF or its epitope on PrP.sup.C are useful in the treatment of prion disease. Pharmacophores of the PrP.sup.C epitope are disclosed as are useful therapeutics and pharmacophores of the PPMF surface which binds PrP.sup.C. Animals resistant to prion disease are taught as are genes for producing such animals. Assay systems are disclosed which use PPMF to amplify PrP.sup.Sc is a sample being tested.

Dependencies are inferred from the claim text.