Stock Tg(PRNP*)K6M16Pcg

FESA Number


Strain Name


Mutation Strain Type


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
466 Fatal familial insomnia
157 941 Huntington disease-like 1
280 397 Familial Alzheimer-like prion disease
282 166 Inherited Creutzfeldt-Jakob disease


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


Orphanet Categories

Rare neurologic disease




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