Stock
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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