- 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
STOCK Prnptm1Cwe Tg(Pcp2-Prnp)E330Zbz/Cnrm
Status | Available to order |
EMMA ID | EM:01824 |
International strain name | STOCK Prnptm1Cwe Tg(Pcp2-Prnp)E330Zbz/Cnrm |
Alternative name | E330 |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(Pcp2-Prnp)E330Zbz, |
Gene/Transgene symbol | Tg(Pcp2-Prnp)E330Zbz |
Information from provider
Provider | Eckhard Flechsig |
Provider affiliation | Institute of Virology and Immunbiology, University Würzburg |
Genetic information | Mouse prion protein deletion 32-121 under control of the Pcp2/L7 promoter. |
Phenotypic information | Purkinje cell degeneration. |
Breeding history | Founder was crossed with Prnp knock-out mice (C57BL/6 x 129/Sv) and hemizygous mice were kept in the presence of one C57BL/6 wild-type Prnp allele. |
References |
|
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)
Literature references
- Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia.;Flechsig Eckhard, Hegyi Ivan, Leimeroth Rainer, Zuniga Armando, Rossi Daniela, Cozzio Antonio, Schwarz Petra, Rülicke Thomas, Götz Jürgen, Aguzzi Adriano, Weissmann Charles, ;2003;The EMBO journal;22;3095-101; 12805223
INFRAFRONTIER® and European Mouse Mutant Archive - EMMA® are registered trademarks at the European Union Intellectual Property Office (EUIPO).