- 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 |
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 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)
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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