Strain Data Sheet

RBRC00437

Strain Information

Image
BRC No.RBRC00437
TypeTargeted Mutation CongenicCartagena
SpeciesMus musculus
Strain nameB6.129P2-Prnp<tm1Ito>/Rbrc
Former Common namePrnp-/- mouse, Prion protein (PrP) Knockout mice
H-2 Haplotype
ES Cell lineE14 [129P2/OlaHsd]
Background strainC57BL/6JJcl
Appearanceblack [a/a B/B C/C]
Strain developmentDeveloped by Drs. Takashi Yokoyama, National Institute of Animal Health and Shigeyoshi Itohara, RIKEN BSI in 2001.C57BL/6J background.
Strain descriptionPrP, the prion protein, plays a central role in the pathogenesis of transmissible neurodegenerative disorders such as scrapie or BSE. Homozygous mutant mice show the absence of cellular isoform of prion protein. Aged homozygous mice exhibit tremor and ataxia.
Colony maintenanceSibling Mating (Homozygote x Homozygote)Aged mice exhibit tremor and ataxia.
References
In vivo conversion of cellular prion protein to pathogenic isoforms, as monitored by conformation-specific antibodies.
Yokoyama T, Kimura K M, Ushiki Y, Yamada S, Morooka A, Nakashiba T, Sassa T, Itohara S
J. Biol. Chem., 276, 11265-11271 (2001). 11152682

Prions prevent neuronal cell-line death.
Kuwahara C, Takeuchi A M, Nishimura T, Haraguchi K, Kubosaki A, Matsumoto Y, Saeki K, Matsumoto Y, Yokoyama T, Itohara S, Onodera T
Nature, 400(6741):225-226 (1999). 10421360

Health Report

Examination Date / Room / Rack

Gene

Gene SymbolGene NameChr.Allele SymbolAllele NameCommon NamesPromoterDiseases Related to This Gene
PrnPprion protein2PrnPtargeted mutation 1, Shigeyoshi Itohara
  • fatal familial insomnia(DisGeNET, MedGEN)

  • Gerstmann-Straussler-Scheinker syndrome(DisGeNET, MedGEN)

  • Huntington disease-like 1(MedGEN)
    • more 3 Diseases
  • inherited Creutzfeldt-Jakob disease(MedGEN)

  • kuru, susceptibility to(MedGEN)

  • spongiform encephalopathy with neuropsychiatric features(MedGEN)
    • neoneomycin resistance gene (E. coli)2mouse phosphoglycerate kinase promoter (PGK promoter)
  • fatal familial insomnia(DisGeNET, MedGEN)

  • Gerstmann-Straussler-Scheinker syndrome(DisGeNET, MedGEN)

  • Huntington disease-like 1(MedGEN)
    • more 3 Diseases
  • inherited Creutzfeldt-Jakob disease(MedGEN)

  • kuru, susceptibility to(MedGEN)

  • spongiform encephalopathy with neuropsychiatric features(MedGEN)

    • Phenotype

    Annotation by Mammalian phenotyhpe ontology
  • ataxia(MP:0001393)

  • tremors(MP:0000745)
    • Detailed phenotype data

    Ordering Information

    Donor DNAmouse phosphoglycerate kinase promoter (PGK promoter), E. coli neo, mouse prion genomic DNA
    Research applicationMouse Models for Human Disease
    Specific Term and ConditionsThe RECIPIENT of BIOLOGICAL RESOURCE shall obtain a prior written consent on use of it from the DEPOSITOR. In publishing the research results obtained by use of the BIOLOGICAL RESOURCE, a citation of the following literature(s) designated by the DEPOSITOR is requested. Nature, 400(6741):225-226 (1999).
    DepositorTakashi Onodera (The University of Tokyo)
    Strain Statusan icon for Frozen embryosFrozen embryos
    an icon for Frozen spermFrozen sperm
    Strain AvailabilityRecovered litters from cryopreserved embryos (2 to 4 months)
    Cryopreserved sperm (within 1 month)
    Cryopreserved embryos (within 1 month)
    Additional Info.Necessary documents for ordering:
    1. Approval form (Japanese / English)
    2. Order form (Japanese / English)
    3. Category I MTA: MTA for distribution with RIKEN BRC (Japanese / English)
    4. Acceptance of responsibility for living modified organism (Japanese / English)

    Genotyping protocol -PCR-

    BRC mice in Publications

    Nakato G, Hase K, Suzuki M, Kimura M, Ato M, Hanazato M, Tobiume M, Horiuchi M, Atarashi R, Nishida N, Watarai M, Imaoka K, Ohno H.
    Cutting Edge: Brucella abortus exploits a cellular prion protein on intestinal M cells as an invasive receptor.
    J Immunol 189(4) 1540-4(2012) 22772447