- 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
STOCK Prnptm1Cwe Tg(Eno2-Prnp)#Aag/Cnrm
Status | Available to order |
EMMA ID | EM:01837 |
International strain name | STOCK Prnptm1Cwe Tg(Eno2-Prnp)#Aag/Cnrm |
Alternative name | B6,129-TgN[NSE]Prnp ZH1/ZH1 |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(Eno2-Prnp)#Aag, |
Gene/Transgene symbol | Tg(Eno2-Prnp)#Aag |
Information from provider
Provider | Adriano Aguzzi |
Provider affiliation | Univ.Hosp.Zurich/Inst.Neuropathology |
Genetic information | Prnp expression under neuron-specific enolase 2 promoter (predominantly on neurons). |
Breeding history | Heterozygous mice were crossed: F1. F1 transgene-positive NSE-Prnp mice were crossed with 129/Sv x C57BL/6. If all offspring mice were transgene-positive, the F1 generation was considerer to be homozygous: 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
- Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC.;Radovanovic Ivan, Braun Nathalie, Giger Olivier T, Mertz Kirsten, Miele Gino, Prinz Marco, Navarro Beatriz, Aguzzi Adriano, ;2005;The Journal of neuroscience : the official journal of the Society for Neuroscience;25;4879-88; 15888663
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