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

FESA Number


Strain Name

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

Mutation Strain Type


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
466 Fatal familial insomnia
157 941 Huntington disease-like 1
280 397 Familial Alzheimer-like prion disease
282 166 Inherited Creutzfeldt-Jakob disease


Displaying 41 - 50 of 226 Stock Publications
Pubmed ID Authors Title Journal
11259483 Rosenmann H Prion protein with an E200K mutation displays properties similar to those of the cellular isoform PrP(C). J Neurochem (2 001) 76:1654-62
17379700 Spinner DS CpG oligodeoxynucleotide-enhanced humoral immune response and production of antibodies to prion protein PrPSc in mice immunized with 139A scrapie-associated fibrils. J Leukoc Biol (2 007) 81:1374-85
21980526 Turnbaugh JA The N-terminal, polybasic region is critical for prion protein neuroprotective activity. PLoS One (2 011) 6:e25675
7719349 Whittington MA Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein. Nat Genet (1 995) 9:197-201
21212268 Anaya ZE Recovery of small infectious PrP(res) aggregates from prion-infected cultured cells. J Biol Chem (2 011) 286:8141-8
11950500 Boudinot E Respiratory function in mice lacking or overexpressing the prion protein. Neurosci Lett (2 002) 323:89-92
21775587 Cissé M Ablation of cellular prion protein does not ameliorate abnormal neural network activity or cognitive dysfunction in the J20 line of human amyloid precursor protein transgenic mice. J Neurosci (2 011) 31:10427-31
17569776 Dong J Doppel induces degeneration of cerebellar Purkinje cells independently of Bax. Am J Pathol (2 007) 171:599-607
18374587 Gavín R Fibrillar prion peptide PrP(106-126) treatment induces Dab1 phosphorylation and impairs APP processing and Abeta production in cortical neurons. Neurobiol Dis (2 008) 30:243-54
21909267 Iken S Th2-polarised PrP-specific transgenic T-cells confer partial protection against murine scrapie. PLoS Pathog (2 011) 7:e1002216


Orphanet Categories

Rare neurologic disease



Genetic Status


Background Strain Name


Background Strain MGI ID



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