Stock

FESA Number

1974

Strain Name

129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH

Mutation Strain Type

Attribution

Gene/Allele Information

Allele Name Allele MGI ID Gene Name Gene MGI ID Chromosome
Prnp<tm1Cwe> MGI:1888773 Prnp MGI:97769 2

Phenotypic Description

The octameric repeat region of the mouse prion protein was replaced with the hexameric repeat region from the domestic chicken. The mice express mouse PrP at wild-type levels with correct membrane location and orientation but have an altered metal binding region. The mice have been generated to get a better understanding of prion diseases such as CJD in humans.

Orphanet Disorders

OrphaNet ID Name Url
356 Gerstmann-Straussler-Scheinker syndrome http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=356
466 Fatal familial insomnia http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=466
157 941 Huntington disease-like 1 http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=157941
280 397 Familial Alzheimer-like prion disease http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=280397
282 166 Inherited Creutzfeldt-Jakob disease http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=282166

Publications

Displaying 181 - 190 of 226 Stock Publications
Pubmed ID Authors Title Journal
20395435 Seelig DM Pathogenesis of chronic wasting disease in cervidized transgenic mice. Am J Pathol (2 010) 176:2785-97
16519879 Thais ME Synaptosomal glutamate release and uptake in mice lacking the cellular prion protein. Brain Res (2 006) 1075:13-9
8030944 Weissmann C PrP-deficient mice are resistant to scrapie. Ann N Y Acad Sci (1 994) 724:235-40
21871438 Young R Generation of Sprn-regulated reporter mice reveals gonadic spatial expression of the prion-like protein Shadoo in mice. Biochem Biophys Res Commun (2 011) 412:752-6
20394048 Benvegnù S Neurodevelopmental expression and localization of the cellular prion protein in the central nervous system of the mouse. J Comp Neurol (2 010) 518:1879-91
8100741 Büeler H Mice devoid of PrP are resistant to scrapie. Cell (1 993) 73:1339-47
11333913 Crozet C Efficient transmission of two different sheep scrapie isolates in transgenic mice expressing the ovine PrP gene. J Virol (2 001) 75:5328-34
24667414 Friedman-Levi Y Genetic prion disease: no role for the immune system in disease pathogenesis? Hum Mol Genet (2 014) 23:4134-41
8845956 Herms JW Patch-clamp analysis of synaptic transmission to cerebellar purkinje cells of prion protein knockout mice. Eur J Neurosci (1 995) 7:2508-12
9414161 Klein MA A crucial role for B cells in neuroinvasive scrapie. Nature (1 997) 390:687-90

Pages

Orphanet Categories

Rare neurologic disease

EMMA ID

EM:02421

Genetic Status

GMO

Background Strain Name

129

Background Strain MGI ID

MGI:2160041

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