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 161 - 170 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 19100703 | Heikenwalder M | Lymphotoxin-dependent prion replication in inflammatory stromal cells of granulomas. | Immunity (2 008) 29:998-1008 |
| 11369504 | Klamt F | Imbalance of antioxidant defense in mice lacking cellular prion protein. | Free Radic Biol Med (2 001) 30:1137-44 |
| 17245437 | Li A | Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125. | EMBO J (2 007) 26:548-58 |
| 17056541 | Martínez del Hoyo G | Prion protein expression by mouse dendritic cells is restricted to the nonplasmacytoid subsets and correlates with the maturation state. | J Immunol (2 006) 177:6137-42 |
| 15970215 | Nico PB | Altered behavioural response to acute stress in mice lacking cellular prion protein. | Behav Brain Res (2 005) 162:173-81 |
| 15888663 | Radovanovic I | Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC. | J Neurosci (2 005) 25:4879-88 |
| 21777560 | Seelig DM | Chronic wasting disease prion trafficking via the autonomic nervous system. | Am J Pathol (2 011) 179:1319-28 |
| 8698234 | Telling GC | Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. | Genes Dev (1 996) 10:1736-50 |
| 16574930 | Weise J | Deletion of cellular prion protein results in reduced Akt activation, enhanced postischemic caspase-3 activation, and exacerbation of ischemic brain injury. | Stroke (2 006) 37:1296-300 |
| 22093825 | Young R | Expression of the prion-like protein Shadoo in the developing mouse embryo. | Biochem Biophys Res Commun (2 011) 416:184-7 |
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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