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 81 - 90 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 21483752 | Miranda A | Prion protein expression regulates embryonic stem cell pluripotency and differentiation. | PLoS One (2 011) 6:e18422 |
| 18400886 | Powell AD | Alterations in Ca2+-buffering in prion-null mice: association with reduced afterhyperpolarizations in CA1 hippocampal neurons. | J Neurosci (2 008) 28:3877-86 |
| 21727894 | Safar JG | Conserved properties of human and bovine prion strains on transmission to guinea pigs. | Lab Invest (2 011) 91:1326-36 |
| 15800202 | Stewart RS | Neurodegenerative illness in transgenic mice expressing a transmembrane form of the prion protein. | J Neurosci (2 005) 25:3469-77 |
| 18316717 | Wadsworth JD | Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. | Proc Natl Acad Sci U S A (2 008) 105:3885-90 |
| 18840415 | Xikota JC | Mild cognitive deficits associated to neocortical microgyria in mice with genetic deletion of cellular prion protein. | Brain Res (2 008) 1241:148-56 |
| 12456643 | Asante EA | BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. | EMBO J (2 002) 21:6358-66 |
| 8917559 | Brandner S | Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system. | Proc Natl Acad Sci U S A (1 996) 93:13148-51 |
| 12876477 | Coitinho AS | Cellular prion protein ablation impairs behavior as a function of age. | Neuroreport (2 003) 14:1375-9 |
| 18066056 | Falsig J | A versatile prion replication assay in organotypic brain slices. | Nat Neurosci (2 008) 11:109-17 |
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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