- 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;129S7-Prnptm1Cwe Tg(Alb1-Prnp)01Cwe/CweCnrm
Status | Available to order |
EMMA ID | EM:00176 |
International strain name | B6;129S7-Prnptm1Cwe Tg(Alb1-Prnp)01Cwe/CweCnrm |
Alternative name | Tg01/Alb |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(Alb1-Prnp)01Cwe, |
Gene/Transgene symbol | Tg(Alb1-Prnp)01Cwe |
Information from provider
Provider | Adriano Aguzzi |
Provider affiliation | Univ.Hosp.Zurich/Inst.Neuropathology |
Genetic information | Introduction of half genomic Prnp (PrP) transgene driven by albumin promoter (copy number 20). |
Phenotypic information | Hepatocyte-specific expression of Prnp (PrP) on Prnp-/- background. Homozygous breeding. |
References |
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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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