STOCK Prnp^tm1Cwe Tg(PrnpD177NM128V)G1Rchi/Cnrm

Status	Available to order
EMMA ID	EM:06144
Citation information	RRID:IMSR_EM:06144 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information.
International strain name	STOCK Prnp^tm1Cwe Tg(PrnpD177NM128V)G1Rchi/Cnrm
Alternative name	Tg(CJD-G1)/Prnp0/0
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Prnp^tm1Cwe, Tg(PrnpD177NM128V)G1Rchi
Gene/Transgene symbol	Prnp, Tg(PrnpD177NM128V)G1Rchi

Information from provider

Provider	Roberto Chiesa
Provider affiliation	Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri
Genetic information	Transgenic mice expressing the mouse homolog (D177N/V128) of the D178N/V129 mutation in the human prion protein gene, linked to a genetic form of Creutzfeldt-Jakob disease (CJD178).
Phenotypic information	This mouse line expresses mouse PrP D177N/V128 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 Prnp-/- mice (EM:00158) for two generations to obtain hemizygous transgenic mice with the disrupted Prnp gene. Tg(CJD-G1+/-),Prnp-/- were intercrossed to obtain Tg(CJD-G1+/+), Prnp-/-mice. The homozygous mice were intercrossed and maintained inbred.
References	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 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
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)

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

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
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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STOCK Prnptm1Cwe Tg(Prnp*D177N*M128V)G1Rchi/Cnrm