Stock

FESA Number

1974

Strain Name

129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH

Mutation Strain Type

Attribution

Gene/Allele Information

Allele Name Allele MGI ID Gene Name Gene MGI ID Chromosome
Prnp<tm1Cwe> MGI:1888773 Prnp MGI:97769 2

Phenotypic Description

The octameric repeat region of the mouse prion protein was replaced with the hexameric repeat region from the domestic chicken. The mice express mouse PrP at wild-type levels with correct membrane location and orientation but have an altered metal binding region. The mice have been generated to get a better understanding of prion diseases such as CJD in humans.

Orphanet Disorders

OrphaNet ID Name Url
356 Gerstmann-Straussler-Scheinker syndrome http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=356
466 Fatal familial insomnia http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=466
157 941 Huntington disease-like 1 http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=157941
280 397 Familial Alzheimer-like prion disease http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=280397
282 166 Inherited Creutzfeldt-Jakob disease http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=282166

Publications

Displaying 211 - 220 of 226 Stock Publications
Pubmed ID Authors Title Journal
7913762 Weissmann C Susceptibility to scrapie in mice is dependent on PrPC. Philos Trans R Soc Lond B Biol Sci (1 994) 343:431-3
17612503 Yun SW beta-Amyloid deposition and prion infection in adult primary brain cell long-term culture model. Biochem Biophys Res Commun (2 007) 360:520-4
21406608 Benvegnù S Developmental influence of the cellular prion protein on the gene expression profile in mouse hippocampus. Physiol Genomics (2 011) 43:711-25
1373228 Büeler H Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature (1 992) 356:577-82
22619325 Daude N Knockout of the prion protein (PrP)-like Sprn gene does not produce embryonic lethality in combination with PrP(C)-deficiency. Proc Natl Acad Sci U S A (2 012) 109:9035-40
16952355 Frigg R The prion protein is neuroprotective against retinal degeneration in vivo. Exp Eye Res (2 006) 83:1350-8
11300727 Herms JW Prion protein affects Ca2+-activated K+ currents in cerebellar purkinje cells. Neurobiol Dis (2 001) 8:324-30
12684540 Korth C Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A (2 003) 100:4784-9
8637886 Lledo PM Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. Proc Natl Acad Sci U S A (1 996) 93:2403-7
19664595 Mays CE Enhancement of protein misfolding cyclic amplification by using concentrated cellular prion protein source. Biochem Biophys Res Commun (2 009) 388:306-10

Pages

Orphanet Categories

Rare neurologic disease

EMMA ID

EM:02421

Genetic Status

GMO

Background Strain Name

129

Background Strain MGI ID

MGI:2160041

Contact/Order

For more information or to order stocks, Contact us