B6.Cg-Prnp^tm1Cwe Tg(Prnp-PG14)1Dah/Cnrm

Status	Available to order
EMMA ID	EM:12881
International strain name	B6.Cg-Prnp^tm1Cwe Tg(Prnp-PG14)1Dah/Cnrm
Alternative name	Tg(PG14-A3+/-)/Prnp0/0
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Prnp^tm1Cwe, 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	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
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)

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

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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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^*

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Practical information

Example health report
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EMMA conditions
Legally binding conditions for the transfer

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B6.Cg-Prnptm1Cwe Tg(Prnp-PG14)1Dah/Cnrm