- 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*D177N*M128V)A21Rchi/Cnrm
Status | Available to order |
EMMA ID | EM:12880 |
International strain name | B6.Cg-Prnptm1Cwe Tg(Prnp*D177N*M128V)A21Rchi/Cnrm |
Alternative name | Tg(CJD-A21+/-)/Prnp0/0 |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(Prnp*D177N*M128V)A21Rchi, |
Gene/Transgene symbol | Tg(Prnp*D177N*M128V)A21Rchi |
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/V128 mutation that in humans is linked to a genetic form of Creutzfeldt-Jakob disease (CJD178) and the epitope for anti-PrP monoclonal antibody 3F4, under the control of the mouse Prnp (PrP) gene promoter. |
Phenotypic information | Homozygous:Neurological disease with cognitive and motor dysfunction, and sleep abnormalities. Neurological signs, including kyphosis, foot clasp reflex, abnormal posture, become overt at approximately 140 days and mice reach a terminal stage at approximately 280 days (Dossena et al., Neuron 60, 598–609, 2008)Heterozygous:Neurological disease with cognitive and motor dysfunction, and sleep abnormalities. Neurological signs, including kyphosis, foot clasp reflex, become overt at approximately 450 days and mice reach a terminal stage at approximately 700 days (Dossena et al., Neuron 60, 598–609, 2008). |
Breeding history | The Tg(CJD-A21+/-)/Prnp+/+ founder (C57BL/6 x CBA) was backcrossed with C57BL/6J-Prnp0/0 mice (EMMA strain EM:01723). |
References |
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Homozygous fertile | not known |
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)
Literature references
- Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model.;Dossena Sara, Imeri Luca, Mangieri Michela, Garofoli Anna, Ferrari Loris, Senatore Assunta, Restelli Elena, Balducci Claudia, Fiordaliso Fabio, Salio Monica, Bianchi Susanna, Fioriti Luana, Morbin Michela, Pincherle Alessandro, Marcon Gabriella, Villani Flavio, Carli Mirjana, Tagliavini Fabrizio, Forloni Gianluigi, Chiesa Roberto, ;2008;Neuron;60;598-609; 19038218
- Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC α(2)δ-1 Subunit.;Senatore Assunta, Colleoni Simona, Verderio Claudia, Restelli Elena, Morini Raffaella, Condliffe Steven B, Bertani Ilaria, Mantovani Susanna, Canovi Mara, Micotti Edoardo, Forloni Gianluigi, Dolphin Annette C, Matteoli Michela, Gobbi Marco, Chiesa Roberto, ;2012;Neuron;74;300-13; 22542184
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