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 191 - 200 of 226 Stock Publications
Pubmed ID Authors Title Journal
22447932 Liang J Cellular prion protein regulates its own ?-cleavage through ADAM8 in skeletal muscle. J Biol Chem (2 012) 287:16510-20
12914944 Mata X Unexpected high testis-specific transcriptional activity of the cyclin T1 promoter in transgenic mice. FEBS Lett (2 003) 549:163-6
10378511 Nishida N A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Lab Invest (1 999) 79:689-97
18638557 Ratté S Hippocampal bursts caused by changes in NMDA receptor-dependent excitation in a mouse model of variant CJD. Neurobiol Dis (2 008) 32:96-104
22542184 Senatore A Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC ?(2)?-1 Subunit. Neuron (2 012) 74:300-13
9030645 Tobler I Sleep and sleep regulation in normal and prion protein-deficient mice. J Neurosci (1 997) 17:1869-79
8407244 Weissmann C Role of the PrP gene in transmissible spongiform encephalopathies. Intervirology (1 993) 35:164-75
19766638 Young R The prion or the related Shadoo protein is required for early mouse embryogenesis. FEBS Lett (2 009) 583:3296-300
21334441 Benvegnù S Aged PrP null mice show defective processing of neuregulins in the peripheral nervous system. Mol Cell Neurosci (2 011) 47:28-35
8790598 Büeler H High prion and PrPSc levels but delayed onset of disease in scrapie-inoculated mice heterozygous for a disrupted PrP gene. Mol Med (1 994) 1:19-30

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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