Strain Data Sheet

RBRC01130

Strain Information

Image
BRC No.RBRC01130
TypeTargeted MutationCartagena
SpeciesMus musculus
Strain nameB6.129P2-Ugt8a<tm1Pop>/Rbrc
Former Common nameCeramide galactosyltransferase (CGT)-deficient mice
H-2 Haplotype
ES Cell lineBK4 [129P2/OlaHsd]
Background strain
Appearanceblack [a/a B/B C/C]
Strain developmentDeveloped by Dr. Brain Popko, University of North Carolina in 1996.
Strain descriptionCGT-deficient mice fail to make galactolipid galactocerebroside and its sulfated derivative that are normal myelin constitutents. Mutant mice exhibit tremors, ataxia, progressive hindlib paralysis and vaculoe formation in ventral spinal cord due to abnormal myelin sheath.B6.129P2-Ugt8a<tm1Pop>. Ceramide galactosyltransferase, CGT is a key enzyme in the biosynthesis of galactocerebroside, GalC. Homozygous mutant mice for CGT gene show tremor, ataxia progressive hind limb paralysis and extensiv vacuolation of the ventral spinal cord.
Colony maintenanceSibling Mating
References
Myelination in the absence of galactocerebroside and sulfatide: normal structure with abnormal function and regional instability.
Coetzee T, Fujita N, Dupree J, Shi R, Blight A, Suzuki K, Suzuki K, Popko B
Cell, 86, 209-219 (1996). 8706126

[Galactosylceramide synthase (CGT) knockout mouse].
Suzuki K
蛋白質 核酸 酵素, 43,2318-2324 (1998). 9883654

Kidney lipids in galactosylceramide synthase-deficient mice. Absence of galactosylsulfatide and compensatory increase in more polar sulfoglycolipids.
Tadano-Aritomi K, Hikita T, Fujimoto H, Suzuki K, Motegi K, Ishizuka I
J. Lipid Res., 41, 1237-1243 (2000). 10946011

Requirement of seminolipid in spermatogenesis revealed by UDP-galactose: Ceramide galactosyltransferase-deficient mice.
Fujimoto H, Tadano-Aritomi K, Tokumasu A, Ito K, Hikita T, Suzuki K, Ishizuka I
J. Biol. Chem., 275, 22623-22626 (2000). 10801776

[Functional analysis of glycolipids in galactosylceramide synthase(CGT)-deficient mice: arrest of male meiosis and compensation of renal function].
Fujimoto H, Tadano-Aritomi K, Ishizuka I, Suzuki K
蛋白質 核酸 酵素, 46, 45-54 (2001). 11193331

Health Report

Examination Date / Room / Rack

Gene

Gene SymbolGene NameChr.Allele SymbolAllele NameCommon NamesPromoterDiseases Related to This Gene
Ugt8
MGI:109522		UDP-glucuronosyltransferase 83Ugt8<tm1Pop>
MGI:2183312		targeted mutation 1, Brian Popko
neo neomycin resistance gene (E. coli)3

Phenotype

Annotation by Mammalian phenotyhpe ontology
  • abnormal Purkinje cell morphology(MP:0000877)

  • abnormal head movements(MP:0000436)

  • abnormal lipid level(MP:0001547)

  • abnormal myelination(MP:0000920)

  • abnormal nerve conduction(MP:0005403)
    • more 7 phenotypes
  • ataxia(MP:0001393)

  • decreased body weight(MP:0001262)

  • hindlimb paralysis(MP:0000755)

  • immune system phenotype(MP:0005387)

  • nervous system phenotype(MP:0003631)

  • premature death(MP:0002083)

  • tremors(MP:0000745)
    • Detailed phenotype data

    Ordering Information

    Donor DNAmouse Ceramide galactosyltransferase (CGT) genomic DNA, E. coli neo
    Research applicationCell Biology Research
    Neurobiology Research
    Specific Term and ConditionsNo specific terms and conditions. (The DEPOSITOR waives its own rights under any patents, intellectual property, or other proprietary rights with respect to the results to be obtained by use of the BIOLOGICAL RESOURCE.)
    DepositorBrian Popko (University of Chicago)
    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. 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