- 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.Cg-Prnptm1Cwe Tg(Prnp*M128*D177N)FFI-10Rchi/Cnrm
Status | Available to order |
EMMA ID | EM:09930 |
International strain name | B6.Cg-Prnptm1Cwe Tg(Prnp*M128*D177N)FFI-10Rchi/Cnrm |
Alternative name | Tg(FFI-10+/+)/Prnp0/0 |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(Prnp*M128*D177N)FFI-10Rchi, Prnptm1Cwe |
Gene/Transgene symbol | Tg(Prnp*M128*D177N)FFI-10Rchi, Prnp |
Information from provider
Provider | Roberto Chiesa |
Provider affiliation | Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri |
Genetic information | Transgenic mice expressing mouse prion protein (moPrP) carrying the D177N substitution that in humans is linked to fatal familial insomnia, under the control of the moPrP gene (Prnp) promoter. |
Phenotypic information | Homozygous:Neurological disease with sleep abnormalities, cognitive and motor dysfunction. Neurological signs become overt at approximately 300 days and mice reach a terminal stage at approximately 700 days (Bouybayoune et al., PLoS Pathog 11(4): e1004796, 2015).Heterozygous:Mild neurological signs. Mice live as long as nontransgenic controls (Bouybayoune et al., PLoS Pathog 11(4): e1004796, 2015). |
Breeding history | The Tg(FFI+/-)/Prnp+/+ founder was backcrossed with C57BL/6J/Prnp0/0 mice (EMMA strain EM:01723). After backcrossing for 10 generations, Tg(FFI+/+)Prnp0/0 mice were intercrossed to produce Tg(FFI+/+)/Prnp0/0 mice. Homozygous mice are maintained by intercrossing. |
References |
|
Homozygous fertile | yes |
Homozygous viable | yes |
Homozygous matings required | no |
Immunocompromised | not known |
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
- Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.;Bouybayoune Ihssane, Mantovani Susanna, Del Gallo Federico, Bertani Ilaria, Restelli Elena, Comerio Liliana, Tapella Laura, Baracchi Francesca, Fernández-Borges Natalia, Mangieri Michela, Bisighini Cinzia, Beznoussenko Galina V, Paladini Alessandra, Balducci Claudia, Micotti Edoardo, Forloni Gianluigi, Castilla Joaquín, Fiordaliso Fabio, Tagliavini Fabrizio, Imeri Luca, Chiesa Roberto, ;2015;PLoS pathogens;11;e1004796; 25880443
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