B6.Cg-Prnptm1Cwe Tg(Prnp*D177N*M128V)A21Rchi/Cnrm

Status

Available to order

EMMA IDEM:12880
International strain nameB6.Cg-Prnptm1Cwe Tg(Prnp*D177N*M128V)A21Rchi/Cnrm
Alternative nameTg(CJD-A21+/-)/Prnp0/0
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolPrnptm1Cwe, Tg(Prnp*D177N*M128V)A21Rchi
Gene/Transgene symbolPrnp, Tg(Prnp*D177N*M128V)A21Rchi

Information from provider

ProviderRoberto Chiesa
Provider affiliationNeuroscience, Istituto di Ricerche Farmacologiche Mario Negri
Genetic informationTransgenic 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 informationHomozygous:
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 historyThe Tg(CJD-A21+/-)/Prnp+/+ founder (C57BL/6 x CBA) was backcrossed with C57BL/6J-Prnp0/0 mice (EMMA strain EM:01723).
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
Homozygous fertilenot known
Homozygous viableyes
Homozygous matings requiredno
Immunocompromisednot known

Information from EMMA

Archiving centreCNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy

Disease and phenotype information

MGI allele-associated human disease models

Orphanet associated rare diseases, based on orthologous gene matching

MGI phenotypes (allele matching)
  • 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
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

  • 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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Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

  • Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

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Practical information

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