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 171 - 180 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 19738901 | Baumann F | Functionally relevant domains of the prion protein identified in vivo. | PLoS One (2 009) 4:e6707 |
| 11782965 | Brown DR | Lack of prion protein expression results in a neuronal phenotype sensitive to stress. | J Neurosci Res (2 002) 67:211-24 |
| 18580573 | Crecelius AC | The brain proteome profile is highly conserved between Prnp-/- and Prnp+/+ mice. | Neuroreport (2 008) 19:1027-31 |
| 16177294 | Fontes P | Absence of evidence for the participation of the macrophage cellular prion protein in infection with Brucella suis. | Infect Immun (2 005) 73:6229-36 |
| 11546838 | Heppner FL | Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies. | Science (2 001) 294:178-82 |
| 17989297 | Kleene R | Prion protein regulates glutamate-dependent lactate transport of astrocytes. | J Neurosci (2 007) 27:12331-40 |
| 17251426 | Li A | N-terminally deleted forms of the prion protein activate both Bax-dependent and Bax-independent neurotoxic pathways. | J Neurosci (2 007) 27:852-9 |
| 21924230 | Martin GR | Endogenous prion protein attenuates experimentally induced colitis. | Am J Pathol (2 011) 179:2290-301 |
| 15767405 | Nikles D | Circumventing tolerance to the prion protein (PrP): vaccination with PrP-displaying retrovirus particles induces humoral immune responses against the native form of cellular PrP. | J Virol (2 005) 79:4033-42 |
| 19855845 | Rangel A | Regulation of GABA(A) and glutamate receptor expression, synaptic facilitation and long-term potentiation in the hippocampus of prion mutant mice. | PLoS One (2 009) 4:e7592 |
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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