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 21 - 30 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 9333232 | Hill AF | The same prion strain causes vCJD and BSE. | Nature (1 997) 389:448-50, 526 |
| 17241158 | Krebs B | Cellular prion protein modulates the intracellular calcium response to hydrogen peroxide. | J Neurochem (2 007) 100:358-67 |
| 17618696 | Lobão-Soares B | Cellular prion protein regulates the motor behaviour performance and anxiety-induced responses in genetically modified mice. | Behav Brain Res (2 007) 183:87-94 |
| 15215178 | McLennan NF | Prion protein accumulation and neuroprotection in hypoxic brain damage. | Am J Pathol (2 004) 165:227-35 |
| 20161712 | Oliveira-Martins JB | Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations. | PLoS One (2 010) 5:e9107 |
| 10521590 | Roesler R | Normal inhibitory avoidance learning and anxiety, but increased locomotor activity in mice devoid of PrP(C). | Brain Res Mol Brain Res (1 999) 71:349-53 |
| 23903654 | Sonati T | The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein. | Nature (2 013) 501:102-6 |
| 22745483 | Turnbaugh JA | The N-terminal, polybasic region of PrP(C) dictates the efficiency of prion propagation by binding to PrP(Sc). | J Neurosci (2 012) 32:8817-30 |
| 10550328 | White AR | Prion protein-deficient neurons reveal lower glutathione reductase activity and increased susceptibility to hydrogen peroxide toxicity. | Am J Pathol (1 999) 155:1723-30 |
| 22252492 | Zhang Y | Multiple effects of the cellular prion protein on tooth development. | Int J Dev Biol (2 011) 55:953-60 |
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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