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 141 - 150 of 226 Stock Publications
Pubmed ID Authors Title Journal
17703189 Watts JC The CNS glycoprotein Shadoo has PrP(C)-like protective properties and displays reduced levels in prion infections. EMBO J (2 007) 26:4038-50
18562311 Yoshikawa D Dominant-negative effects of the N-terminal half of prion protein on neurotoxicity of prion protein-like protein/doppel in mice. J Biol Chem (2 008) 283:24202-11
15262264 Barmada S GFP-tagged prion protein is correctly localized and functionally active in the brains of transgenic mice. Neurobiol Dis (2 004) 16:527-37
9523587 Brown DR Effects of copper on survival of prion protein knockout neurons and glia. J Neurochem (1 998) 70:1686-93
22956830 Cortes CJ Rapamycin delays disease onset and prevents PrP plaque deposition in a mouse model of Gerstmann-Sträussler-Scheinker disease. J Neurosci (2 012) 32:12396-405
12805223 Flechsig E Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia. EMBO J (2 003) 22:3095-101
23238082 Hartmann CA High levels of cellular prion protein improve astrocyte development. FEBS Lett (2 013) 587:238-44
18443219 Khosravani H Prion protein attenuates excitotoxicity by inhibiting NMDA receptors. J Cell Biol (2 008) 181:551-65
17510630 Li A Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice. EMBO J (2 007) 26:2777-85
17270731 Mallucci GR Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron (2 007) 53:325-35

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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