Inventors:
Stanley B. Prusiner - San Francisco CA
Michael R. Scott - San Francisco CA
Glenn C. Telling - San Francisco CA
Assignee:
The Regents of the University of California - Oakland CA
International Classification:
C12N 1509, C12N 509, A61K 4900
Abstract:
The invention includes an artificial PrP gene, a transgenic animal containing a PrP gene of another animal or the artificial PrP gene, a hybrid non-human mammal with an ablated endogenous prion protein gene and exogenous prion protein gene, assay methodology which uses the animals to detect pathogenic prions in a sample and standardized prion preparation used in the assay. The genome of a host animal (such as a mouse), is manipulated so that the animal is rendered susceptible to infection with prions which normally would infect only a genetically diverse test animal (such as human, cow or sheep). A PrP gene of the host is preferably manipulated to include a mutation which matches a mutation which causes prion disease in the genetically diverse mammal. Pathogenic prions in a sample can be detected by injecting the sample to be tested into a mammal of the invention which has been genetically manipulated so as to be susceptible to infection from prions in the sample. Mammals which are not inoculated with the sample and others inoculated with a standardized prion preparation of the invention are used as controls in the assay to detect prions in samples which cause diseases.