Compositions and methods for the study and diagnosis of prion diseases

摘要

The present invention presents novel characteristics of a transmembrane form of PrP (^CtmPrP) that is retained in the endoplasmic reticulum, and contains an uncleaved, N-terminal signal peptide as well as a C-terminal glycolipid anchor. The invention also identifies a mutant form of PrP that is synthesized exclusively with the ^CtmPrP topology as well as related nucleic acid sequences and transfected cells and mammalian animals. Compositions and methods for the further study of ^CtmPrP and mutant PrP, and their role in the causation and diagnosis of prion related diseases are further embodied in the invention.