- 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;129S7-Prnptm1Cwe/CweCnrm
Status | Available to order |
EMMA ID | EM:00158 |
Citation information | RRID:IMSR_EM:00158 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 | B6;129S7-Prnptm1Cwe/CweCnrm |
Alternative name | Prp-KO |
Strain type | Targeted Mutant Strains : Knock-out |
Allele/Transgene symbol | Prnptm1Cwe |
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 |
|
Information from EMMA
Archiving centre | CNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy |
Animals used for archiving | homozygous males, wild-type females |
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)
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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