Detecting prions in a sample and prion preparation and transgeni

Multicellular living organisms and unmodified parts thereof and – Nonhuman animal

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4351723, 424 91, C12N 1509, C12N 509, A61K 4900

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active

057929017

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. For example, Creutzfeldt Jakob Disease (CJD) is a fatal neurodegenerative disease of humans caused by prions.

REFERENCES:
Goldfarb et al, "Fatal Familial Insomnia and Familial Creutzfeldt-Jakob Disease: Disease Phenotype Determined by a DNA Polymorphism," Science (1992) 258:806-808.
Goldmann et al., "Two Alleles of a Neural Protein Gene Linked to Scrapie in Sheep," Proc. Natl. Acad. Sci. USA (1990) 87:2476-2480.
Goldmann et al., "Different Forms of the Bovine PrP Gene Have Five or Six Copies of a Short, G-C Rich Element within the protein-coding Exon," J. Gen. Virol. (1991) 72:201-204.
Harris et al., "A Prion-like Protein from Chicken Brain Copurifies with an Acetylcholine Receptor-Inducing Activity," Proc. Natl. Acad. Sci. USA (1991) 88:7664-7668.
Hasty, P., et al., "Introduction of a subtle mutation into the Hox-2.6 locus in embryonic stem cells", Nature (1991) 350:243-246.
Healy et al., "Creutzfeldt-Jakob Disease After Pituitary Gonadotrophins: The Prion is the Problem," BMJ (1993) 307:517-518.
Hecker et al., "Replication of Distinct Scrapie Prion Isolates is Region Specific in Brains of Transgenic Mice and Hamsters," Genes Dev. (1992) 6:1213-1228.
Hsaio et al., "Linkage of a Prion Protein Missense Variant to Gerstmann-Straussler Syndrome," Nature (1989) 383:342-345.
Hsaio et al., "A Prion Protein Variant in a Family with the Telencephalic Form of Gerstmann-Strussler-Scheinker Syndrome," Neurology (1991) 41:681-684.
Hsiao et al., "Inherited Human Prion Diseases," Neurology (1990) 40:1820-1827.
Kascsak, R.J., et al., "Mouse Polyclonal and Monoclonal Antibody to Scrapie-Associated Fibril Proteins," J. Virol. (1987) 61(12):3688-3693.
Koch et al., "Creutzfeldt-Jakob Disease in a Young Adult with Idiopathic Hypopituitarism," N. Engl. J. Med. (1985) 313:731-733.
Kretzschmar et al., "Molecular Cloning of a Human Prion Protein cDNA," DNA (1986) 5:315-324.
Kretzschmar et al., "Molecular Cloning of a Mink Prion Protein Gene," J.Gen.Virol. (1992) 73:2757-2761.
Lasmezas et al., "Recombinant Human Growth Hormone and Insulin-Like Growth Factor I Induce PRP Gene Expression in PC12 Cell," Biochem. Biophys. Res.Commun. (1993) 196:1163-1169.
Locht et al., "Molecular Cloning and Complete Sequence of Prion Protein cDNA from Mouse Brain Infected with the Scrapie Agent" Proc. Natl. Acad. Sci USA (1986) 83:6372-6376.
Manuelidis et al., "Serial Propagation of Creutzfeldt-Jakob Disease in Guinea Pigs," Proc. Natl. Acad. Sci. USA (1976) 73:223-227.
Manuelidis et al., "Interspecies Transmission of Creutzfeldt-Jakob Disease to Syrian Hamsters with Reference to Clinical Syndromes and Strain of Agent," Proc. Natl. Acad. Sci USA (1978) 75:3432-3436.
McKinley et al, "A Protease-Resistant Protein is a Structural Component of the Scrapie Prion," Cell (1983) 35:57-62.
Medori et al., "Fatal Familial Insomnia, a Prion Disease with a Mutation at Codon 178 of the Prion Protein Gene," N. Engl.J. Med. (1992) 326:444-449.
Muramoto, T., et al., "The Sequential Development of Abnormal Prion Protein Accumulation in Mice with Creuzfeldt-Jakob Disease," Am. J. Pathol. (1992) 146(6):1411-1420.
Nisbet et al., "Creutzfeldt-Jakob Disease in a Second Patient Who Received a Cadaveric Dura mater Graft," J.Am. Med.Assoc. (1989) 261:1118.
Palmer, M.S., et al., "Homozygous Prion Protein Genotype Predisposes to Sporadi Creutzfeldt-Jakob Disease", Nature (1991) 352:340-342.
Patel, "France Reels at Latest Medical Scandal," New Scientist, Jul. 31, 1993, p. 4.
Baker, H.F., et al. "Aminoacid Polymorphism in Human Prion Protein and Age at Death in Inherited Prion Disease," Lancet (1991) 337:1286.
Barry, R.A., et al., "Monoclonal Antibodies to the Cellular and Scrapie Prion Proteins," J. Infect. Dis. (1986) 154(3):518-521.
Basler et al., "Scrapie and Cellular PrP Isoforms Are Encoded by the Same Chromosomal Gene," Cell, (1986) 46:417-28.
Berger, J.R., et al., "Creutzfeldt-Jakob disease in a physician: A review of the disorder in health care workers", Neurology, (1993) 43:205-206.
Bolton et al., "Identification of Protein That Purifies with the Scrapie Prion," Science (1982) 218 :1309-11.
Brown et al., "Friendly Fire` in Medicine: Hormones, Homografts, and Cruetzfeldt-Jakob Disease," Lancet (1992) 340: 24-27.
Buchanan et al., "Mortality, Neoplasia, Creutzfeld-Jakob Disease in Patients Treated with Human Pituitary Growth Hormone in the United Kingdom", BMJ (1991) 302:824-828.
Bueler et al., "Mice Devoid of PrP are Resistant to Scrapie," Cell (1993) 73:1339-1347.
Bueler et al., "Normal Development and Behavior of Mice Lacking the Neuronal Cell-surface PrP Protein," Nature (1992) 356:577-582.
Carlson et al., "Linkage of Protein and Scrapie Incubation Time Genes," Cell (1986) 46:503-511.
Chandler, "Encephaolpathy in Mice Produced by Inoculation with Scrapie Brain Material," Lancet (1961) 1:1378-79.
Cochius et al, "Creutzfeldt-Jakob Disease in a Recipient of Human Pituitary-Derived Gonadotrophin: A Second Case," J. Neurol. Neurosurg. Psychiatry (1992) 55:1094-1095.
Cochius et al., "Creutzfeldt-Jakob Disease in a Recipient of Human Pituitary-Derived Gonadotrophin," Aust. N.Z. J. Med. (1990) 20:592-593.
Collinge et al., "Genetic Predisposition to Latrogenic Creutzfeldt-Jakob Disease," Lancet (1991) 337:1441-1442.
Cousens, S.N., et al., "Geographical distribution of cases of Creutzfeldt-Jakob disease in England and Wales 1970-84", J. Neurol. Neurosurg. Psychiatry (1990) 53:459-465.
Farlie, P.G., et al., "bcl-2 Transgene expression can protect neurons against developmental and induced cell death", Proc. Natl. Acad. Sci. USA (1995) 92:4397-4401.
Gabriel et al., "Molecular Cloning of a Candidate Chicken Prion Protein," Proc. Natl. Acad. Sci. USA (1992) 89:9097-9101.
Gajdusek, D.C., "Unconventional Viruses and the Origin and Disappearance of Kuru," Science (1977) 197:943-960.
Gibbs, Jr. et al., "Creutzfeldt-Jakob Disease Infectivity of Growth Hormone Derived from Human Pituitary Glands," N.Engl. J. Med. (1993) 328:358-359.
Patel, "Placenta Donors to be Screened for Brain Disease," New Scientist, Nov. 20, 1993, p. 10.
Pan, K.M., et al., "Conversion of .beta.-sheets features in the formation of the scrapie prion proteins", Proc. Natl. Acad. Sci. USA (1993) 90:10962-10966.
Prusiner et al., "Measurement of the Scrapie Agent Using an Incubation Time Interval Assay," Annals. Neurol. (1982) 11(4):353-358.
Prusiner et al., "Further Purification and Characterization of Scrapie Prions," Biochemistry (1982) 21:6942-50.
Prusiner, S.B., et al., "Scrapie Prions Aggregate to Form Amyloid-like Birefringent Rods," Cell (1983) 35:349-358.
Prusiner et al., "Transgenic Studies Implicate Interactions Between Homologous PrP Isoforms in Scrapie Prion Replication," Cell (1990) 63:673-686.
Prusiner et al., "Molecular Biology of Prion Diseases," Science (1991) 252:1515-1522.
Prusiner et al., "Ablation of the Prion Protein (PrP) Gene in Mice Prevents Scrapie and Facilitates Production of Anti-PrP Antibodies,"

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