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

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 81 - 90 of 226 Stock Publications
Pubmed ID Authors Title Journal
21483752 Miranda A Prion protein expression regulates embryonic stem cell pluripotency and differentiation. PLoS One (2 011) 6:e18422
18400886 Powell AD Alterations in Ca2+-buffering in prion-null mice: association with reduced afterhyperpolarizations in CA1 hippocampal neurons. J Neurosci (2 008) 28:3877-86
21727894 Safar JG Conserved properties of human and bovine prion strains on transmission to guinea pigs. Lab Invest (2 011) 91:1326-36
15800202 Stewart RS Neurodegenerative illness in transgenic mice expressing a transmembrane form of the prion protein. J Neurosci (2 005) 25:3469-77
18316717 Wadsworth JD Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc Natl Acad Sci U S A (2 008) 105:3885-90
18840415 Xikota JC Mild cognitive deficits associated to neocortical microgyria in mice with genetic deletion of cellular prion protein. Brain Res (2 008) 1241:148-56
12456643 Asante EA BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J (2 002) 21:6358-66
8917559 Brandner S Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system. Proc Natl Acad Sci U S A (1 996) 93:13148-51
12876477 Coitinho AS Cellular prion protein ablation impairs behavior as a function of age. Neuroreport (2 003) 14:1375-9
18066056 Falsig J A versatile prion replication assay in organotypic brain slices. Nat Neurosci (2 008) 11:109-17

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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