Strain Data Sheet

RBRC09860

Strain Information

Image
BRC No.RBRC09860
TypeTransgeneCartagena
SpeciesMus musculus
Strain nameB6.B6D2-Tg(Thy1-FUS<*>deltaNLS)1Dit
Former Common namedeltaNLF-FUStg
H-2 Haplotype
ES Cell line
Background strain
Appearance
Strain developmentA human FUS cDNA without a nuclear localization signal was inserted into the pTSC21k vector, which encodes the 8.1 kb murine Thy1.2 expression cassette. This construct was Not I digested to remove vector sequences, co-injected into the pronucleus of fertilized eggs from Slc:BDF1 strain mice. Founders were bred and backcrossed with C57Bl6/J mice to establish transgenic lines.
Strain descriptionA transgenic (tg) mouse overexpressing exogenous FUS without a nuclear localization signal (ΔNLS-FUS) develop progressive spastic motor deficits and neuronal loss in the motor cortex. The ΔNLS-FUS protein was restricted to the cytoplasm and formed ubiquitin/p62-positive aggregates. Endogenous FUS expression, nuclear localization, and splicing activity were not altered, indicating that mislocated FUS is sufficient for proteinopathy. RNA-sequence analysis revealed specific transcriptome alterations, including genes regulating dynein-associated molecules and endoplasmic reticulum stress.
Colony maintenanceHeterozygote x Wild-type [or Crossing to C57BL/6JJcl]
References
Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.
Shiihashi G, Ito D, Yagi T, Nihei Y, Ebine T, Suzuki N
Brain, 139(Pt 9):2380-94 (2016). 27368346

Health Report

Examination Date / Room / Rack

Gene

Gene SymbolGene NameChr.Allele SymbolAllele NameCommon NamesPromoterDiseases Related to This Gene
FUS
MGI:1353633		fused in sarcomaUNFUS
  • amyotrophic lateral sclerosis type 6(MedGEN)

  • tremor, hereditary essential, 4(MedGEN)

    • Phenotype

    Annotation by Mammalian phenotyhpe ontology
  • abnormal motor coordination/balance(MP:0001516)

  • abnormal stride length(MP:0012690)

  • abnormal survival(MP:0010769)

  • athetotic walking movements(MP:0001527)

  • limb grasping(MP:0001513)
    • more 3 phenotypes
  • nervous system inclusion bodies(MP:0014062)

  • neuronal cytoplasmic inclusions(MP:0011975)

  • tremors(MP:0000745)
    • Detailed phenotype data

    Ordering Information

    Donor DNAmouse Thy-1 promoter, mouse Thy-1 exon 3,4 genomic DNA, human FUS cDNA without a nuclear localization signal
    Research application
    Specific Term and ConditionsIn publishing the research results obtained by use of the BIOLOGICAL RESOURCE, a citation of the following literature(s) designated by the DEPOSITOR is requested. Brain 2016 Sep;139(Pt 9):2380-94. doi: 10.1093/brain/aww161. Epub 2016 Jun 30.
    DepositorDaisuke Ito (Keio University)
    Strain Statusan icon for Live miceLive mice
    an icon for Frozen embryosFrozen embryos
    an icon for Frozen spermFrozen sperm
    Strain AvailabilityRecovery and QC required prior to distribution
    Cryopreserved sperm (within 1 month)
    Additional Info.Necessary documents for ordering:
    1. Order form (Japanese / English)
    2. Category I MTA: MTA for distribution with RIKEN BRC (Japanese / English)
    3. Acceptance of responsibility for living modified organism (Japanese / English)

    Genotyping protocol -PCR-

    BRC mice in Publications

    No Data