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 101 - 110 of 226 Stock Publications
Pubmed ID Authors Title Journal
15082172 Asante EA Pathogenic human prion protein rescues PrP null phenotype in transgenic mice. Neurosci Lett (2 004) 360:33-6
20098419 Bremer J Axonal prion protein is required for peripheral myelin maintenance. Nat Neurosci (2 010) 13:310-8
8035877 Collinge J Prion protein is necessary for normal synaptic function. Nature (1 994) 370:295-7
23133383 Falsig J Prion pathogenesis is faithfully reproduced in cerebellar organotypic slice cultures. PLoS Pathog (2 012) 8:e1002985
25692227 Guitart K Prion protein regulates glutathione metabolism and neural glutamate and cysteine uptake via excitatory amino acid transporter 3. J Neurochem (2 015) 133:558-71
16517717 Jouvin-Marche E Overexpression of cellular prion protein induces an antioxidant environment altering T cell development in the thymus. J Immunol (2 006) 176:3490-7
23345215 Lemaire-Vieille C Ataxia with cerebellar lesions in mice expressing chimeric PrP-Dpl protein. J Neurosci (2 013) 33:1391-9
16730679 Maglio LE Role of cellular prion protein on LTP expression in aged mice. Brain Res (2 006) 1097:11-8
11274428 Montrasio F B lymphocyte-restricted expression of prion protein does not enable prion replication in prion protein knockout mice. Proc Natl Acad Sci U S A (2 001) 98:4034-7
15229245 Prinz M Intrinsic resistance of oligodendrocytes to prion infection. J Neurosci (2 004) 24:5974-81

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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