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 41 - 50 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 11259483 | Rosenmann H | Prion protein with an E200K mutation displays properties similar to those of the cellular isoform PrP(C). | J Neurochem (2 001) 76:1654-62 |
| 17379700 | Spinner DS | CpG oligodeoxynucleotide-enhanced humoral immune response and production of antibodies to prion protein PrPSc in mice immunized with 139A scrapie-associated fibrils. | J Leukoc Biol (2 007) 81:1374-85 |
| 21980526 | Turnbaugh JA | The N-terminal, polybasic region is critical for prion protein neuroprotective activity. | PLoS One (2 011) 6:e25675 |
| 7719349 | Whittington MA | Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein. | Nat Genet (1 995) 9:197-201 |
| 21212268 | Anaya ZE | Recovery of small infectious PrP(res) aggregates from prion-infected cultured cells. | J Biol Chem (2 011) 286:8141-8 |
| 11950500 | Boudinot E | Respiratory function in mice lacking or overexpressing the prion protein. | Neurosci Lett (2 002) 323:89-92 |
| 21775587 | Cissé M | Ablation of cellular prion protein does not ameliorate abnormal neural network activity or cognitive dysfunction in the J20 line of human amyloid precursor protein transgenic mice. | J Neurosci (2 011) 31:10427-31 |
| 17569776 | Dong J | Doppel induces degeneration of cerebellar Purkinje cells independently of Bax. | Am J Pathol (2 007) 171:599-607 |
| 18374587 | Gavín R | Fibrillar prion peptide PrP(106-126) treatment induces Dab1 phosphorylation and impairs APP processing and Abeta production in cortical neurons. | Neurobiol Dis (2 008) 30:243-54 |
| 21909267 | Iken S | Th2-polarised PrP-specific transgenic T-cells confer partial protection against murine scrapie. | PLoS Pathog (2 011) 7:e1002216 |
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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