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 151 - 160 of 226 Stock Publications
Pubmed ID Authors Title Journal
17494993 Nicolas O Bcl-2 overexpression delays caspase-3 activation and rescues cerebellar degeneration in prion-deficient mice that overexpress amino-terminally truncated prion. FASEB J (2 007) 21:3107-17
21559407 Quaglio E Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction. PLoS One (2 011) 6:e19339
18498440 Schmalzbauer R Evidence for an association of prion protein and sphingolipid-mediated signaling. J Neurochem (2 008) 106:1459-70
7553876 Telling GC Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell (1 995) 83:79-90
22163178 Watts JC Protease-resistant prions selectively decrease Shadoo protein. PLoS Pathog (2 011) 7:e1002382
22307640 You H A? neurotoxicity depends on interactions between copper ions, prion protein, and N-methyl-D-aspartate receptors. Proc Natl Acad Sci U S A (2 012) 109:1737-42
17245436 Baumann F Lethal recessive myelin toxicity of prion protein lacking its central domain. EMBO J (2 007) 26:538-47
14511115 Brown DR Prion protein expression modulates neuronal copper content. J Neurochem (2 003) 87:377-85
16843609 Coulpier M Axotomy-induced motoneuron death is delayed in mice overexpressing PrPc. Neuroscience (2 006) 141:1827-34
10985358 Flechsig E Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice. Neuron (2 000) 27:399-408

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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