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.