- 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(Prnp)a19Cwe/CweCnrm
Status | Available to order |
EMMA ID | EM:00180 |
International strain name | B6;129S7-Prnptm1Cwe Tg(Prnp)a19Cwe/CweCnrm |
Alternative name | Tga19 |
Strain type | Targeted Mutant Strains : Knock-out |
Allele/Transgene symbol | Prnptm1Cwe, Tg(Prnp)a19Cwe |
Gene/Transgene symbol | Prnp, Tg(Prnp)a19Cwe |
Information from provider
Provider | Adriano Aguzzi |
Provider affiliation | Univ.Hosp.Zurich/Inst.Neuropathology |
Genetic information | Reintroduction of Prnp (PrP) constructs on Prnp-/- background. Introduction of half genomic PrP construct (phgPrP; copy number 30). 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)
MGI phenotypes (gene matching)
- impaired fertilization / MGI
- abnormal spleen morphology / MGI
- tremors / MGI
- abnormal cerebral cortex morphology / MGI
- abnormal hippocampus morphology / MGI
- abnormal olfactory bulb morphology / MGI
- abnormal thalamus morphology / MGI
- abnormal cerebellum morphology / MGI
- Purkinje cell degeneration / MGI
- decreased Purkinje cell number / MGI
- abnormal cerebellar molecular layer / MGI
- thin cerebellar molecular layer / MGI
- abnormal retina morphology / MGI
- ataxia / MGI
- hypoactivity / MGI
- impaired coordination / MGI
- reduced long term potentiation / MGI
- abnormal sleep pattern / MGI
- abnormal body temperature homeostasis / MGI
- male infertility / MGI
- premature death / MGI
- abnormal muscle physiology / MGI
- abnormal brain morphology / MGI
- no abnormal phenotype detected / MGI
- gliosis / MGI
- abnormal CNS synaptic transmission / MGI
- neurodegeneration / MGI
- spongiform encephalopathy / MGI
- decreased vertical activity / MGI
- abnormal inhibitory postsynaptic potential / MGI
- abnormal inhibitory postsynaptic currents / MGI
- no phenotypic analysis / MGI
- increased neuron apoptosis / MGI
- neuron degeneration / MGI
- astrocytosis / MGI
- abnormal voluntary movement / MGI
- nervous system phenotype / MGI
- abnormal nervous system morphology / MGI
- impaired acrosome reaction / MGI
- abnormal behavior / MGI
- abnormal neuronal precursor proliferation / MGI
- decreased susceptibility to prion infection / MGI
- increased susceptibility to prion infection / MGI
- behavior/neurological phenotype / MGI
- immune system phenotype / MGI
- teratozoospermia / MGI
- brain vacuoles / MGI
- abnormal brain white matter morphology / MGI
- abnormal hippocampus CA1 region morphology / MGI
- decreased neuron number / MGI
- abnormal neuron differentiation / MGI
- abnormal neuron proliferation / MGI
- decreased brain copper level / MGI
- enlarged brain ventricles / MGI
- cerebellum atrophy / MGI
- altered susceptibility to prion infection / MGI
Literature references
- Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.;Fischer M, Rülicke T, Raeber A, Sailer A, Moser M, Oesch B, Brandner S, Aguzzi A, Weissmann C, ;1996;The EMBO journal;15;1255-64; 8635458
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