Stock

FESA Number

1975

Strain Name

Tg(PRNP*)K6M16Pcg

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

Overexpression of transgenic kudu PrPC in brain and other tissues, in order to confer increased susceptibility to TSE diseases, including BSE, compared to wild-type mice. Tg(KuPrP)K6M16 mice are hemizygous for the transgene.

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 61 - 70 of 226 Stock Publications
Pubmed ID Authors Title Journal
23349890 Cohen E Snord 3A: a molecular marker and modulator of prion disease progression. PLoS One (2 013) 8:e54433
19038218 Dossena S Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model. Neuron (2 008) 60:598-609
19846876 Gourdain P Adoptive transfer of T lymphocytes sensitized against the prion protein attenuates prion invasion in scrapie-infected mice. J Immunol (2 009) 183:6619-28
18462346 Isaacs JD The cellular prion protein is preferentially expressed by CD4+ CD25+ Foxp3+ regulatory T cells. Immunology (2 008) 125:313-9
24431459 Kurt TD Prion transmission prevented by modifying the ?2-?2 loop structure of host PrPC. J Neurosci (2 014) 34:1022-7
17987132 Lötscher M Induced prion protein controls immune-activated retroviruses in the mouse spleen. PLoS One (2 007) 2:e1158
24038599 Michel B Complement protein C3 exacerbates prion disease in a mouse model of chronic wasting disease. Int Immunol (2 013) 25:697-702
21195746 Petrosino S Alteration of the endocannabinoid system in mouse brain during prion disease. Neuroscience (2 011) 177:292-7
19209230 Rutishauser D The comprehensive native interactome of a fully functional tagged prion protein. PLoS One (2 009) 4:e4446
15671025 Stewart RS A transmembrane form of the prion protein is localized in the Golgi apparatus of neurons. J Biol Chem (2 005) 280:15855-64

Pages

Orphanet Categories

Rare neurologic disease

EMMA ID

EM:02539

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