- 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-PG14)1Dah/Cnrm
Status | Available to order |
EMMA ID | EM:12881 |
International strain name | B6.Cg-Prnptm1Cwe Tg(Prnp-PG14)1Dah/Cnrm |
Alternative name | Tg(PG14-A3+/-)/Prnp0/0 |
Strain type | Targeted Mutant Strains : Knock-out |
Allele/Transgene symbol | Prnptm1Cwe, Tg(Prnp-PG14)1Dah |
Gene/Transgene symbol | Prnp, Tg(Prnp-PG14)1Dah |
Information from provider
Provider | Roberto Chiesa |
Provider affiliation | Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri IRCCS |
Additional owner | Dr. David A. Harris, Boston University School of Medicine, Department of Biochemistry, Boston, US |
Genetic information | Transgenic mice expressing mouse prion protein (Prnp or mPrP) carrying a nine-octapeptide repeats insertion that in humans is linked to Gerstmann-Sträussler-Scheinker (GSS) syndrome and the epitope for anti-PrP monoclonal antibody 3F4, under the control of the mouse Prnp gene promoter. |
Phenotypic information | Homozygous:Neurological disease with cerebellar ataxia. Neurological signs, including kyphosis, foot clasp reflex, abnormal posture, become overt at approximately 65 days and mice reach a terminal stage at approximately 140 days.Heterozygous:Neurological disease with cerebellar ataxia. Neurological signs, including kyphosis, foot clasp reflex, abnormal posture, become overt at approximately 240 days and mice reach a terminal stage at approximately 450 days. |
Breeding history | The Tg(PG14-A3+/-)/Prnp+/+ founder (C57BL/6 x CBA) was backcrossed first with (129 x C57BL/6J)-Prnp0/0 (EMMA strain EM:00158) then with C57BL/6J-backcrossed Prnp0/0 (EMMA strain EM:01723) mice. |
References |
|
Homozygous fertile | no |
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
- Neurological illness in transgenic mice expressing a prion protein with an insertional mutation.;Chiesa R, Piccardo P, Ghetti B, Harris D A, ;1998;Neuron;21;1339-51; 9883727
- Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation.;Chiesa R, Drisaldi B, Quaglio E, Migheli A, Piccardo P, Ghetti B, Harris D A, ;2000;Proceedings of the National Academy of Sciences of the United States of America;97;5574-9; 10805813
- Primary myopathy and accumulation of PrPSc-like molecules in peripheral tissues of transgenic mice expressing a prion protein insertional mutation.;Chiesa R, Pestronk A, Schmidt R E, Tourtellotte W G, Ghetti B, Piccardo P, Harris D A, ;2001;Neurobiology of disease;8;279-88; 11300723
- 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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