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 11 - 20 of 226 Stock Publications
Pubmed ID Authors Title Journal
19747526 Rial D Cellular prion protein modulates age-related behavioral and neurochemical alterations in mice. Neuroscience (2 009) 164:896-907
19568430 Singh A Prion protein (PrP) knock-out mice show altered iron metabolism: a functional role for PrP in iron uptake and transport. PLoS One (2 009) 4:e6115
18815152 Tsutsui S Absence of the cellular prion protein exacerbates and prolongs neuroinflammation in experimental autoimmune encephalomyelitis. Am J Pathol (2 008) 173:1029-41
21957261 Westergard L A nine amino acid domain is essential for mutant prion protein toxicity. J Neurosci (2 011) 31:14005-17
17947689 Zabel MD Stromal complement receptor CD21/35 facilitates lymphoid prion colonization and pathogenesis. J Immunol (2 007) 179:6144-52
25654651 Altmeppen HC The sheddase ADAM10 is a potent modulator of prion disease. Elife (2 015) 4:
22222374 Bertuchi FR PrPC displays an essential protective role from oxidative stress in an astrocyte cell line derived from PrPC knockout mice. Biochem Biophys Res Commun (2 012) 418:27-32
20649983 Chadi S Brain transcriptional stability upon prion protein-encoding gene invalidation in zygotic or adult mouse. BMC Genomics (2 010) 11:448
20610764 Devanathan V Cellular form of prion protein inhibits Reelin-mediated shedding of Caspr from the neuronal cell surface to potentiate Caspr-mediated inhibition of neurite outgrowth. J Neurosci (2 010) 30:9292-305
21439331 Gadotti VM Depressive-like behaviour of mice lacking cellular prion protein. Behav Brain Res (2 012) 227:319-23

Pages

Orphanet Categories

Rare neurologic disease

EMMA ID

EM:02539

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