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 11 - 20 of 226 Stock Publications
Pubmed ID Authors Title Journal
19747526 Rial D Cellular prion protein modulates age-related behavioral and neurochemical alterations in mice. Neuroscience (2 009) 164:896-907
19568430 Singh A Prion protein (PrP) knock-out mice show altered iron metabolism: a functional role for PrP in iron uptake and transport. PLoS One (2 009) 4:e6115
18815152 Tsutsui S Absence of the cellular prion protein exacerbates and prolongs neuroinflammation in experimental autoimmune encephalomyelitis. Am J Pathol (2 008) 173:1029-41
21957261 Westergard L A nine amino acid domain is essential for mutant prion protein toxicity. J Neurosci (2 011) 31:14005-17
17947689 Zabel MD Stromal complement receptor CD21/35 facilitates lymphoid prion colonization and pathogenesis. J Immunol (2 007) 179:6144-52
25654651 Altmeppen HC The sheddase ADAM10 is a potent modulator of prion disease. Elife (2 015) 4:
22222374 Bertuchi FR PrPC displays an essential protective role from oxidative stress in an astrocyte cell line derived from PrPC knockout mice. Biochem Biophys Res Commun (2 012) 418:27-32
20649983 Chadi S Brain transcriptional stability upon prion protein-encoding gene invalidation in zygotic or adult mouse. BMC Genomics (2 010) 11:448
20610764 Devanathan V Cellular form of prion protein inhibits Reelin-mediated shedding of Caspr from the neuronal cell surface to potentiate Caspr-mediated inhibition of neurite outgrowth. J Neurosci (2 010) 30:9292-305
21439331 Gadotti VM Depressive-like behaviour of mice lacking cellular prion protein. Behav Brain Res (2 012) 227:319-23


Orphanet Categories

Rare neurologic disease



Genetic Status


Background Strain Name


Background Strain MGI ID



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