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 161 - 170 of 226 Stock Publications
Pubmed ID Authors Title Journal
19100703 Heikenwalder M Lymphotoxin-dependent prion replication in inflammatory stromal cells of granulomas. Immunity (2 008) 29:998-1008
11369504 Klamt F Imbalance of antioxidant defense in mice lacking cellular prion protein. Free Radic Biol Med (2 001) 30:1137-44
17245437 Li A Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125. EMBO J (2 007) 26:548-58
17056541 Martínez del Hoyo G Prion protein expression by mouse dendritic cells is restricted to the nonplasmacytoid subsets and correlates with the maturation state. J Immunol (2 006) 177:6137-42
15970215 Nico PB Altered behavioural response to acute stress in mice lacking cellular prion protein. Behav Brain Res (2 005) 162:173-81
15888663 Radovanovic I Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC. J Neurosci (2 005) 25:4879-88
21777560 Seelig DM Chronic wasting disease prion trafficking via the autonomic nervous system. Am J Pathol (2 011) 179:1319-28
8698234 Telling GC Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev (1 996) 10:1736-50
16574930 Weise J Deletion of cellular prion protein results in reduced Akt activation, enhanced postischemic caspase-3 activation, and exacerbation of ischemic brain injury. Stroke (2 006) 37:1296-300
22093825 Young R Expression of the prion-like protein Shadoo in the developing mouse embryo. Biochem Biophys Res Commun (2 011) 416:184-7

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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