- 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 |
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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)
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
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