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 51 - 60 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 22100763 | Kudo W | Cellular prion protein is essential for oligomeric amyloid-?-induced neuronal cell death. | Hum Mol Genet (2 012) 21:1138-44 |
| 16339028 | Lopes MH | Interaction of cellular prion and stress-inducible protein 1 promotes neuritogenesis and neuroprotection by distinct signaling pathways. | J Neurosci (2 005) 25:11330-9 |
| 17433806 | Meotti FC | Involvement of cellular prion protein in the nociceptive response in mice. | Brain Res (2 007) 1151:84-90 |
| 11239719 | Pereira GS | Changes in cortical and hippocampal ectonucleotidase activities in mice lacking cellular prion protein. | Neurosci Lett (2 001) 301:72-4 |
| 11179214 | Rossi D | Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain. | EMBO J (2 001) 20:694-702 |
| 15893468 | Spudich A | Aggravation of ischemic brain injury by prion protein deficiency: role of ERK-1/-2 and STAT-1. | Neurobiol Dis (2 005) 20:442-9 |
| 19285121 | Vidal C | Early dysfunction of central 5-HT system in a murine model of bovine spongiform encephalopathy. | Neuroscience (2 009) 160:731-43 |
| 21641403 | Williams SK | Neuroprotective effects of the cellular prion protein in autoimmune optic neuritis. | Am J Pathol (2 011) 178:2823-31 |
| 19243016 | Arantes C | Prion protein and its ligand stress inducible protein 1 regulate astrocyte development. | Glia (2 009) 57:1439-49 |
| 19666958 | Boy J | Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3. | Hum Mol Genet (2 009) 18:4282-95 |
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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