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 171 - 180 of 226 Stock Publications
Pubmed ID Authors Title Journal
19738901 Baumann F Functionally relevant domains of the prion protein identified in vivo. PLoS One (2 009) 4:e6707
11782965 Brown DR Lack of prion protein expression results in a neuronal phenotype sensitive to stress. J Neurosci Res (2 002) 67:211-24
18580573 Crecelius AC The brain proteome profile is highly conserved between Prnp-/- and Prnp+/+ mice. Neuroreport (2 008) 19:1027-31
16177294 Fontes P Absence of evidence for the participation of the macrophage cellular prion protein in infection with Brucella suis. Infect Immun (2 005) 73:6229-36
11546838 Heppner FL Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies. Science (2 001) 294:178-82
17989297 Kleene R Prion protein regulates glutamate-dependent lactate transport of astrocytes. J Neurosci (2 007) 27:12331-40
17251426 Li A N-terminally deleted forms of the prion protein activate both Bax-dependent and Bax-independent neurotoxic pathways. J Neurosci (2 007) 27:852-9
21924230 Martin GR Endogenous prion protein attenuates experimentally induced colitis. Am J Pathol (2 011) 179:2290-301
15767405 Nikles D Circumventing tolerance to the prion protein (PrP): vaccination with PrP-displaying retrovirus particles induces humoral immune responses against the native form of cellular PrP. J Virol (2 005) 79:4033-42
19855845 Rangel A Regulation of GABA(A) and glutamate receptor expression, synaptic facilitation and long-term potentiation in the hippocampus of prion mutant mice. PLoS One (2 009) 4:e7592

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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