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 111 - 120 of 226 Stock Publications
Pubmed ID Authors Title Journal
16198494 Sakurai-Yamashita Y Female-specific neuroprotection against transient brain ischemia observed in mice devoid of prion protein is abolished by ectopic expression of prion protein-like protein. Neuroscience (2 005) 136:281-7
23851597 Striebel JF Prion protein and susceptibility to kainate-induced seizures: genetic pitfalls in the use of PrP knockout mice. Prion (2 013) 7:280-5
25058617 Wang M The cellular prion protein negatively regulates phagocytosis and cytokine expression in murine bone marrow-derived macrophages. PLoS One (2 014) 9:e102785
22927985 Yamaguchi Y Biological and biochemical characterization of mice expressing prion protein devoid of the octapeptide repeat region after infection with prions. PLoS One (2 012) 7:e43540
24086135 Asante EA Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein. PLoS Pathog (2 013) 9:e1003643
22529900 Bribián A Role of the cellular prion protein in oligodendrocyte precursor cell proliferation and differentiation in the developing and adult mouse CNS. PLoS One (2 012) 7:e33872
8734907 Colling SB Hippocampal slices from prion protein null mice: disrupted Ca(2+)-activated K+ currents. Neurosci Lett (1 996) 209:49-52
15607951 Fioriti L The neurotoxicity of prion protein (PrP) peptide 106-126 is independent of the expression level of PrP and is not mediated by abnormal PrP species. Mol Cell Neurosci (2 005) 28:165-76
17504807 Hajj GN Cellular prion protein interaction with vitronectin supports axonal growth and is compensated by integrins. J Cell Sci (2 007) 120:1915-26
22948149 Kang HE Characterization of conformation-dependent prion protein epitopes. J Biol Chem (2 012) 287:37219-32

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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