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 51 - 60 of 226 Stock Publications
Pubmed ID Authors Title Journal
22100763 Kudo W Cellular prion protein is essential for oligomeric amyloid-?-induced neuronal cell death. Hum Mol Genet (2 012) 21:1138-44
16339028 Lopes MH Interaction of cellular prion and stress-inducible protein 1 promotes neuritogenesis and neuroprotection by distinct signaling pathways. J Neurosci (2 005) 25:11330-9
17433806 Meotti FC Involvement of cellular prion protein in the nociceptive response in mice. Brain Res (2 007) 1151:84-90
11239719 Pereira GS Changes in cortical and hippocampal ectonucleotidase activities in mice lacking cellular prion protein. Neurosci Lett (2 001) 301:72-4
11179214 Rossi D Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain. EMBO J (2 001) 20:694-702
15893468 Spudich A Aggravation of ischemic brain injury by prion protein deficiency: role of ERK-1/-2 and STAT-1. Neurobiol Dis (2 005) 20:442-9
19285121 Vidal C Early dysfunction of central 5-HT system in a murine model of bovine spongiform encephalopathy. Neuroscience (2 009) 160:731-43
21641403 Williams SK Neuroprotective effects of the cellular prion protein in autoimmune optic neuritis. Am J Pathol (2 011) 178:2823-31
19243016 Arantes C Prion protein and its ligand stress inducible protein 1 regulate astrocyte development. Glia (2 009) 57:1439-49
19666958 Boy J Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3. Hum Mol Genet (2 009) 18:4282-95


Orphanet Categories

Rare neurologic disease



Genetic Status


Background Strain Name


Background Strain MGI ID



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