B6;129S7-Prnp^tm1Cwe/CweCnrm

Status	Available to order
EMMA ID	EM:00158
International strain name	B6;129S7-Prnp^tm1Cwe/CweCnrm
Alternative name	Prp-KO
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Prnp^tm1Cwe
Gene/Transgene symbol	Prnp

Information from provider

Provider	Adriano Aguzzi
Provider affiliation	Univ.Hosp.Zurich/Inst.Neuropathology
Genetic information	Generation of Prnp (PrP) knock-out mice. Disruption of Prnp allele of ES cells by homologous recombination. A 4.8 kb genomic sequence was replaced by neo gene under the HSV TK promoter. Homozygous breeding.
References	Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein.;Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp H P, DeArmond S J, Prusiner S B, Aguet M, Weissmann C, ;1992;Nature;356;577-82; 1373228

Information from EMMA

Archiving centre	CNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy
Animals used for archiving	homozygous 0, wild-type 0
Breeding at archiving centre	A sub-strain with the same mutation and genetic background is maintained at the repository at the Institut de Transgenose, INTRAGENE, Orleans, France: EM:05303
Stage of embryos	2-cell

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

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein.;Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp H P, DeArmond S J, Prusiner S B, Aguet M, Weissmann C, ;1992;Nature;356;577-82; 1373228
Neuroprotective role of PrPC against kainate-induced epileptic seizures and cell death depends on the modulation of JNK3 activation by GluR6/7-PSD-95 binding.;Carulla Patricia, Bribián Ana, Rangel Alejandra, Gavín Rosalina, Ferrer Isidro, Caelles Carme, Del Río José Antonio, Llorens Franc, ;2011;Molecular biology of the cell;22;3041-54; 21757544
Development of monoclonal antibodies specific for glycated prion protein.;Dvorakova Eva, Prouza Marek, Janouskova Olga, Panigaj Martin, Holada Karel, ;2011;Journal of toxicology and environmental health. Part A;74;1469-75; 22043908
Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers.;Laurén Juha, Gimbel David A, Nygaard Haakon B, Gilbert John W, Strittmatter Stephen M, ;2009;Nature;457;1128-32; 19242475
A proautophagic antiviral role for the cellular prion protein identified by infection with a herpes simplex virus 1 ICP34.5 mutant.;Korom Maria, Wylie Kristine M, Wang Hong, Davis Katie L, Sangabathula Meher S, Delassus Gregory S, Morrison Lynda A, ;2013;Journal of virology;87;5882-94; 23487467
Regulation of GABA(A) and glutamate receptor expression, synaptic facilitation and long-term potentiation in the hippocampus of prion mutant mice.;Rangel Alejandra, Madroñal Noelia, Gruart Agnès, Gavín Rosalina, Llorens Franc, Sumoy Lauro, Torres Juan María, Delgado-García José María, Del Río José Antonio, ;2009;PloS one;4;e7592; 19855845
Absence of the cellular prion protein exacerbates and prolongs neuroinflammation in experimental autoimmune encephalomyelitis.;Tsutsui Shigeki, Hahn Jennifer N, Johnson Trina A, Ali Zenobia, Jirik Frank R, ;2008;The American journal of pathology;173;1029-41; 18815152
Role of the cellular prion protein in oligodendrocyte precursor cell proliferation and differentiation in the developing and adult mouse CNS.;Bribián Ana, Fontana Xavier, Llorens Franc, Gavín Rosalina, Reina Manuel, García-Verdugo José Manuel, Torres Juan María, de Castro Fernando, del Río José Antonio, ;2012;PloS one;7;e33872; 22529900
Involvement of Cellular Prion Protein in α-Synuclein Transport in Neurons.;Urrea Laura, Segura-Feliu Miriam, Masuda-Suzukake Masami, Hervera Arnau, Pedraz Lucas, García Aznar José Manuel, Vila Miquel, Samitier Josep, Torrents Eduard, Ferrer Isidro, Gavín Rosalina, Hagesawa Masato, Del Río José Antonio, ;2018;Molecular neurobiology;55;1847-1860; 28229331
Depressive-like behaviour of mice lacking cellular prion protein.;Gadotti Vinicius M, Bonfield Stephan P, Zamponi Gerald W, ;2012;Behavioural brain research;227;319-23; 21439331
Cellular prion protein protects from inflammatory and neuropathic pain.;Gadotti Vinicius M, Zamponi Gerald W, ;2011;Molecular pain;7;59; 21843375
Prion protein attenuates excitotoxicity by inhibiting NMDA receptors.;Khosravani Houman, Zhang Yunfeng, Tsutsui Shigeki, Hameed Shahid, Altier Christophe, Hamid Jawed, Chen Lina, Villemaire Michelle, Ali Zenobia, Jirik Frank R, Zamponi Gerald W, ;2008;The Journal of cell biology;181;551-65; 18443219

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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 embryos. 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).

More details on pricing and delivery times

Practical information

Genotyping protocol

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
For this strain no provider MTA is needed. Distribution is based on the EMMA conditions only.

EMMA conditions
Legally binding conditions for the transfer

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B6;129S7-Prnptm1Cwe/CweCnrm