- reduced long term potentiation / MGI
- abnormal CNS synaptic transmission / MGI
- abnormal inhibitory postsynaptic potential / MGI
- abnormal inhibitory postsynaptic currents / MGI
- behavior/neurological phenotype / MGI
- decreased brain copper level / MGI
- tremors / MGI
- abnormal cerebellum morphology / MGI
- decreased Purkinje cell number / MGI
B6;129-Prnptm1Cwe Tg(IRF1-Prnp)94Cwe/Cnrm
Status | Available to order |
EMMA ID | EM:04911 |
International strain name | B6;129-Prnptm1Cwe Tg(IRF1-Prnp)94Cwe/Cnrm |
Alternative name | B6,129-Tg94+/+[IRF],Prnp |
Strain type | Targeted Mutant Strains : Knock-out |
Allele/Transgene symbol | Prnptm1Cwe, |
Gene/Transgene symbol | Prnp |
Information from provider
Provider | Adriano Aguzzi |
Provider affiliation | Univ.Hosp.Zurich/Inst.Neuropathology |
Additional owner | Charles Weissmann |
Genetic information | Overexpression of PrP under the IRF1 promoter/Ig heavy chain enhancer. |
Phenotypic information | none |
Breeding history | No backcrossing. Only brother x sister matings. |
References |
|
Homozygous fertile | yes |
Homozygous viable | yes |
Homozygous matings required | no |
Immunocompromised | no |
Information from EMMA
Archiving centre | CNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy |
Disease and phenotype information
Orphanet associated rare diseases, based on orthologous gene matching
- Inherited Creutzfeldt-Jakob disease / Orphanet_282166
- Familial Alzheimer-like prion disease / Orphanet_280397
- Huntington disease-like 1 / Orphanet_157941
- PrP systemic amyloidosis / Orphanet_397606
- Fatal familial insomnia / Orphanet_466
- Gerstmann-Straussler-Scheinker syndrome / Orphanet_356
- Sporadic fatal insomnia / Orphanet_586130
MGI phenotypes (allele matching)
Literature references
- Ectopic expression of prion protein (PrP) in T lymphocytes or hepatocytes of PrP knockout mice is insufficient to sustain prion replication.;Raeber A J, Sailer A, Hegyi I, Klein M A, Rülicke T, Fischer M, Brandner S, Aguzzi A, Weissmann C, ;1999;Proceedings of the National Academy of Sciences of the United States of America;96;3987-92; 10097150
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