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 61 - 70 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 23349890 | Cohen E | Snord 3A: a molecular marker and modulator of prion disease progression. | PLoS One (2 013) 8:e54433 |
| 19038218 | Dossena S | Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model. | Neuron (2 008) 60:598-609 |
| 19846876 | Gourdain P | Adoptive transfer of T lymphocytes sensitized against the prion protein attenuates prion invasion in scrapie-infected mice. | J Immunol (2 009) 183:6619-28 |
| 18462346 | Isaacs JD | The cellular prion protein is preferentially expressed by CD4+ CD25+ Foxp3+ regulatory T cells. | Immunology (2 008) 125:313-9 |
| 24431459 | Kurt TD | Prion transmission prevented by modifying the ?2-?2 loop structure of host PrPC. | J Neurosci (2 014) 34:1022-7 |
| 17987132 | Lötscher M | Induced prion protein controls immune-activated retroviruses in the mouse spleen. | PLoS One (2 007) 2:e1158 |
| 24038599 | Michel B | Complement protein C3 exacerbates prion disease in a mouse model of chronic wasting disease. | Int Immunol (2 013) 25:697-702 |
| 21195746 | Petrosino S | Alteration of the endocannabinoid system in mouse brain during prion disease. | Neuroscience (2 011) 177:292-7 |
| 19209230 | Rutishauser D | The comprehensive native interactome of a fully functional tagged prion protein. | PLoS One (2 009) 4:e4446 |
| 15671025 | Stewart RS | A transmembrane form of the prion protein is localized in the Golgi apparatus of neurons. | J Biol Chem (2 005) 280:15855-64 |
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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