Method of detecting prions in a sample and transgenic animal use

Details Method of detecting prions in a sample and transgenic animal use Method of detecting prions in a sample and transgenic animal use

1995-08-31

1999-06-01

Stanton, Brian R.

Multicellular living organisms and unmodified parts thereof and

Method of using a transgenic nonhuman animal to manufacture...

800 18, C12P 2100

Patent

active

059089695

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 or diagnose a cause of death 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). 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. For example, Creutzfeldt Jakob Disease (CJD) is a fatal neurodegenerative disease of humans caused by prions. The mammals disclosed herein develop the adverse effects of such prions in a relatively short time after being inoculated with human prions.

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