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

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 71 - 80 of 226 Stock Publications
Pubmed ID Authors Title Journal
17062642 Viegas P Junctional expression of the prion protein PrPC by brain endothelial cells: a role in trans-endothelial migration of human monocytes. J Cell Sci (2 006) 119:4634-43
16282497 Windl O Breaking an absolute species barrier: transgenic mice expressing the mink PrP gene are susceptible to transmissible mink encephalopathy. J Virol (2 005) 79:14971-5
19806224 Arsac JN Transmissibility of atypical scrapie in ovine transgenic mice: major effects of host prion protein expression and donor prion genotype. PLoS One (2 009) 4:e7300
8552188 Brandner S Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature (1 996) 379:339-43
12425947 Coitinho AS Decreased hyperlocomotion induced by MK-801, but not amphetamine and caffeine in mice lacking cellular prion protein (PrP(C)). Brain Res Mol Brain Res (2 002) 107:190-4
24574409 Douet JY PrP expression level and sensitivity to prion infection. J Virol (2 014) 88:5870-2
16272297 Grégoire S The murine B cell repertoire is severely selected against endogenous cellular prion protein. J Immunol (2 005) 175:6443-9
19700753 Jeffrey M Prion protein with an insertional mutation accumulates on axonal and dendritic plasmalemma and is associated with distinctive ultrastructural changes. Am J Pathol (2 009) 175:1208-17
19242475 Laurén J Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. Nature (2 009) 457:1128-32
20138835 Lutz J Microdeletions within the hydrophobic core region of cellular prion protein alter its topology and metabolism. Biochem Biophys Res Commun (2 010) 393:439-44

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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