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 21 - 30 of 226 Stock Publications
Pubmed ID Authors Title Journal
9333232 Hill AF The same prion strain causes vCJD and BSE. Nature (1 997) 389:448-50, 526
17241158 Krebs B Cellular prion protein modulates the intracellular calcium response to hydrogen peroxide. J Neurochem (2 007) 100:358-67
17618696 Lobão-Soares B Cellular prion protein regulates the motor behaviour performance and anxiety-induced responses in genetically modified mice. Behav Brain Res (2 007) 183:87-94
15215178 McLennan NF Prion protein accumulation and neuroprotection in hypoxic brain damage. Am J Pathol (2 004) 165:227-35
20161712 Oliveira-Martins JB Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations. PLoS One (2 010) 5:e9107
10521590 Roesler R Normal inhibitory avoidance learning and anxiety, but increased locomotor activity in mice devoid of PrP(C). Brain Res Mol Brain Res (1 999) 71:349-53
23903654 Sonati T The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein. Nature (2 013) 501:102-6
22745483 Turnbaugh JA The N-terminal, polybasic region of PrP(C) dictates the efficiency of prion propagation by binding to PrP(Sc). J Neurosci (2 012) 32:8817-30
10550328 White AR Prion protein-deficient neurons reveal lower glutathione reductase activity and increased susceptibility to hydrogen peroxide toxicity. Am J Pathol (1 999) 155:1723-30
22252492 Zhang Y Multiple effects of the cellular prion protein on tooth development. Int J Dev Biol (2 011) 55:953-60

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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