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 61 - 70 of 226 Stock Publications
Pubmed ID Authors Title Journal
23349890 Cohen E Snord 3A: a molecular marker and modulator of prion disease progression. PLoS One (2 013) 8:e54433
19038218 Dossena S Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model. Neuron (2 008) 60:598-609
19846876 Gourdain P Adoptive transfer of T lymphocytes sensitized against the prion protein attenuates prion invasion in scrapie-infected mice. J Immunol (2 009) 183:6619-28
18462346 Isaacs JD The cellular prion protein is preferentially expressed by CD4+ CD25+ Foxp3+ regulatory T cells. Immunology (2 008) 125:313-9
24431459 Kurt TD Prion transmission prevented by modifying the ?2-?2 loop structure of host PrPC. J Neurosci (2 014) 34:1022-7
17987132 Lötscher M Induced prion protein controls immune-activated retroviruses in the mouse spleen. PLoS One (2 007) 2:e1158
24038599 Michel B Complement protein C3 exacerbates prion disease in a mouse model of chronic wasting disease. Int Immunol (2 013) 25:697-702
21195746 Petrosino S Alteration of the endocannabinoid system in mouse brain during prion disease. Neuroscience (2 011) 177:292-7
19209230 Rutishauser D The comprehensive native interactome of a fully functional tagged prion protein. PLoS One (2 009) 4:e4446
15671025 Stewart RS A transmembrane form of the prion protein is localized in the Golgi apparatus of neurons. J Biol Chem (2 005) 280:15855-64


Orphanet Categories

Rare neurologic disease



Genetic Status


Background Strain Name


Background Strain MGI ID



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