Method of detecting prions in a sample and transgenic animal use

Multicellular living organisms and unmodified parts thereof and – Nonhuman animal

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424 91, 424 92, 435 721, 4351723, 530350, 800DIG1, 800DIG2, 800DIG4, 935 34, 935 53, 935 70, A61K 4900, C12N 500, C12N 1500, C12N 1509

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057637407

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 gene and exogenous prion gene and assay methodology which uses the animals to detect pathogenic prions in a sample or diagnose a cause of death. The artificial gene includes a sequence such that when it is inserted into the genome of a host animal (such as a mouse), 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). The artificial PrP gene may be comprised of a completely artificial polynucleotide sequence. Alternatively, the artificial gene may be comprised of the codon sequence of a host animal with one or more codon substitutions being made wherein the substitutions are preferably corresponding PrP gene codons from a genetically diverse test animal. Pathogenic prions in a sample can be detected by injecting the sample to be tested into a transgenic mouse which includes the different codons of the prion protein gene of the animal (e.g. human) in danger of infection from prions in the sample.

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