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 191 - 200 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 22447932 | Liang J | Cellular prion protein regulates its own ?-cleavage through ADAM8 in skeletal muscle. | J Biol Chem (2 012) 287:16510-20 |
| 12914944 | Mata X | Unexpected high testis-specific transcriptional activity of the cyclin T1 promoter in transgenic mice. | FEBS Lett (2 003) 549:163-6 |
| 10378511 | Nishida N | A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. | Lab Invest (1 999) 79:689-97 |
| 18638557 | Ratté S | Hippocampal bursts caused by changes in NMDA receptor-dependent excitation in a mouse model of variant CJD. | Neurobiol Dis (2 008) 32:96-104 |
| 22542184 | Senatore A | Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC ?(2)?-1 Subunit. | Neuron (2 012) 74:300-13 |
| 9030645 | Tobler I | Sleep and sleep regulation in normal and prion protein-deficient mice. | J Neurosci (1 997) 17:1869-79 |
| 8407244 | Weissmann C | Role of the PrP gene in transmissible spongiform encephalopathies. | Intervirology (1 993) 35:164-75 |
| 19766638 | Young R | The prion or the related Shadoo protein is required for early mouse embryogenesis. | FEBS Lett (2 009) 583:3296-300 |
| 21334441 | Benvegnù S | Aged PrP null mice show defective processing of neuregulins in the peripheral nervous system. | Mol Cell Neurosci (2 011) 47:28-35 |
| 8790598 | Büeler H | High prion and PrPSc levels but delayed onset of disease in scrapie-inoculated mice heterozygous for a disrupted PrP gene. | Mol Med (1 994) 1:19-30 |
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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