Stock

FESA Number

1974

Strain Name

129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH

Mutation Strain Type

Mutation Type Mutation Subtype Strain Type
IMSR - Transgenic IMSR - unclassified

Attribution

Institution name Depositor / Originator Name
Univeristy of Bath Originator Retained
Univeristy of Bath Depositor Retained

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 1 - 10 of 226 Stock Publications
Pubmed ID Authors Title Journal
18358791 Albanese V Evidence for prion protein expression in enteroglial cells of the myenteric plexus of mouse intestine. Auton Neurosci (2 008) 140:17-23
23729591 Beraldo FH Stress-inducible phosphoprotein 1 has unique cochaperone activity during development and regulates cellular response to ischemia via the prion protein. FASEB J (2 013) 27:3594-607
24312586 Campeau JL Early increase and late decrease of purkinje cell dendritic spine density in prion-infected organotypic mouse cerebellar cultures. PLoS One (2 013) 8:e81776
15539455 de Almeida CJ The cellular prion protein modulates phagocytosis and inflammatory response. J Leukoc Biol (2 005) 77:238-46
16945105 Fuhrmann M Loss of the cellular prion protein affects the Ca2+ homeostasis in hippocampal CA1 neurons. J Neurochem (2 006) 98:1876-85
24810941 Hernandez-Rapp J A PrP(C)-caveolin-Lyn complex negatively controls neuronal GSK3? and serotonin 1B receptor. Sci Rep (2 014) 4:4881
18490487 Kranich J Follicular dendritic cells control engulfment of apoptotic bodies by secreting Mfge8. J Exp Med (2 008) 205:1293-302
18590772 Lobão-Soares B Cellular prion protein modulates defensive attention and innate fear-induced behaviour evoked in transgenic mice submitted to an agonistic encounter with the tropical coral snake Oxyrhopus guibei. Behav Brain Res (2 008) 194:129-37
24430187 Mays CE Prion disease tempo determined by host-dependent substrate reduction. J Clin Invest (2 014) 124:847-58
24145514 Nuvolone M SIRP? polymorphisms, but not the prion protein, control phagocytosis of apoptotic cells. J Exp Med (2 013) 210:2539-52

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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