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 11 - 20 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 19747526 | Rial D | Cellular prion protein modulates age-related behavioral and neurochemical alterations in mice. | Neuroscience (2 009) 164:896-907 |
| 19568430 | Singh A | Prion protein (PrP) knock-out mice show altered iron metabolism: a functional role for PrP in iron uptake and transport. | PLoS One (2 009) 4:e6115 |
| 18815152 | Tsutsui S | Absence of the cellular prion protein exacerbates and prolongs neuroinflammation in experimental autoimmune encephalomyelitis. | Am J Pathol (2 008) 173:1029-41 |
| 21957261 | Westergard L | A nine amino acid domain is essential for mutant prion protein toxicity. | J Neurosci (2 011) 31:14005-17 |
| 17947689 | Zabel MD | Stromal complement receptor CD21/35 facilitates lymphoid prion colonization and pathogenesis. | J Immunol (2 007) 179:6144-52 |
| 25654651 | Altmeppen HC | The sheddase ADAM10 is a potent modulator of prion disease. | Elife (2 015) 4: |
| 22222374 | Bertuchi FR | PrPC displays an essential protective role from oxidative stress in an astrocyte cell line derived from PrPC knockout mice. | Biochem Biophys Res Commun (2 012) 418:27-32 |
| 20649983 | Chadi S | Brain transcriptional stability upon prion protein-encoding gene invalidation in zygotic or adult mouse. | BMC Genomics (2 010) 11:448 |
| 20610764 | Devanathan V | Cellular form of prion protein inhibits Reelin-mediated shedding of Caspr from the neuronal cell surface to potentiate Caspr-mediated inhibition of neurite outgrowth. | J Neurosci (2 010) 30:9292-305 |
| 21439331 | Gadotti VM | Depressive-like behaviour of mice lacking cellular prion protein. | Behav Brain Res (2 012) 227:319-23 |
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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