- 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
STOCK Prnptm1Cwe Tg(Prnp*)CDah/Cnrm
Status | Available to order |
EMMA ID | EM:06142 |
International strain name | STOCK Prnptm1Cwe Tg(Prnp*)CDah/Cnrm |
Alternative name | Tg(PG14-C)/Prnp0/0 |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(Prnp*)CDah, |
Gene/Transgene symbol | Tg(Prnp*)CDah |
Information from provider
Provider | Roberto Chiesa |
Provider affiliation | Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri |
Additional owner | David A. Harris, Washington University in St. Louis, St. Louis MO, USA (now Professor and Chair Department of Biochemistry, Boston University School of Medicine, Boston MA, USA) |
Genetic information | Transgenic mice expressing the mouse homolog (PG14) of a 72-amino acid (nine-octapeptide) insertion in the prion protein gene linked to an inherited prion disease. |
Phenotypic information | This mouse line expresses PG14 PrP at low levels (approximately 0.3 fold the endogenous PrP level) and does not develop a neurological illness. |
Breeding history | The transgenic (C57BL/6J x CBA) founder was backcrossed with Prnp0/0 mice (EM:00158) for two generations to obtain hemizygous transgenic mice with the disrupted Prnp gene. Tg(PG14-C+/-)/Prnp0/0 were intercrossed to obtain Tg(PG14-C+/+)/Prnp0/0 mice. The homozygous line is maintained inbred. |
References |
|
Homozygous fertile | yes |
Homozygous viable | yes |
Homozygous matings required | no |
Immunocompromised | no |
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
- 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
- Bax deletion prevents neuronal loss but not neurological symptoms in a transgenic model of inherited prion disease.;Chiesa Roberto, Piccardo Pedro, Dossena Sara, Nowoslawski Lisa, Roth Kevin A, Ghetti Bernardino, Harris David A, ;2005;Proceedings of the National Academy of Sciences of the United States of America;102;238-43; 15618403
- Aggregated, wild-type prion protein causes neurological dysfunction and synaptic abnormalities.;Chiesa Roberto, Piccardo Pedro, Biasini Emiliano, Ghetti Bernardino, Harris David A, ;2008;The Journal of neuroscience : the official journal of the Society for Neuroscience;28;13258-67; 19052217
- 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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