Strain Name:

B6.CE-Galctwi/J

Stock Number:

000845

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Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain CE/J
Generation+F2N8F1N1p
Generation Definitions

Appearance
black, tremors
Related Genotype: a/a Galctwi/Galctwi

black, unaffected
Related Genotype: a/a +/?

Description
The twitcher mouse is a neurological leukodystrophy mutant first observed in 1976 at The Jackson Laboratory. Initially characterized on a mixed C57BL/6J and CE/J background, clinical symptoms were first observed by day 30 and homozygotes did not survive beyond three months of age. (Duchen LW, et. al., 1980) Subsequent backcrosses to C57BL/6J and the generation of a full congenic (> 10 backcrosses) reduced the onset of symptoms to approximately 21 days with death by 40 days. Head tremors and decreased body weight are initial clinical indicators and mice are generally less active than unaffected littermates. Muscle weakness in the hindlimbs is a promiment feature and clinically, the health of the mutants progressively declines until death. There is a significant lack of myelin in the twitcher CNS, along with astrocytic gliosis. The nerves in the PNS are also demyelinated. The mutant CNS and PNS contain multinucleated, periodic acid-Schiff-positive globoid cells. Electron microscopic analysis shows these cells contain paracrystalline inclusions and twisted tubules.

Galactosylceramidase (GALC) is the enzyme responsible for the initial step of galactosylceramide (or galactocerebroside) degradation. Galactocerebroside is one of the most abundant and unique lipid constituents of the myelin sheath and the twitcher mouse is a useful mutant in which to study myelina tion and myelin metabolism. This substrate of the enzyme GALC, however, does not accumulate in tissues of affected mice (or humans). The pathol ogies are believed to result from the abnormal accumulation of the cytotoxic metabolite galactosylsphingosine (psychosine), another substrate of GALC that inhibits protein kinase C, that causes myelin-forming cells of the CNS and PNS to dysfunction and undergo apoptosis. Levels of myelin protein mRNAs are normal through postnatal day 20 but decline after day 25, corresponding to the observed pathological demyelinating changes. The data indicate that specific gene expression during myelination appears normal initially. Astrogliosis in the CNS is initiated prior to the appearance of myelin pathologies (as early as postnatal 15) and GFAP mRNA is highly upregulated after day 20, presumably as a response to demyelination. Cytokines are believed to play a major role in the inflammatory responses associated with the disease course. TNF-alpha and IL-6 in the CNS appear to be induced by the pathological condition; reactive astrocytes and microglia contribute to the pathogenic course in the CNS of these mutants.(Taniike et al., 1998; Kobayashi et al., 1980; Suzuki and Suzuki, 1995; LeVine and Brown, 1997; Matsushima et al., 1994).

Development
The twitcher mutation (Galctwi) arose spontaneously at The Jackson Laboratory in 1976 in the CE/J strain when that inbred was at generation F69. The mutant subline was sibling bred for 2 generations and then the mutation was moved onto the C57BL/6J background by means of ovarian transplantation cross-intercross breeding. The female host carrying the homozygous mutation was crossed to a C57BL/6J male and the obligate heterozygote offspring were intercrossed to produce more homozygous females for ovarian transplantation. In 1983 C57BL/6J females were bred with heterozygous males at generation +F2N8F1 to generate embryos for cryopreservation.

Control Information

  Control
   Untyped from the colony
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Galc
000978   B10.A/(5R)SgSn-Galctwi-2J/J
003613   BXD32/TyJ-Galctwi-5J/J
View Strains carrying other alleles of Galc     (2 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Krabbe Disease
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Galctwi/Galctwi

        B6.CE-Galctwi/J
  • mortality/aging
  • premature death
    • lifespan of mutants is 45.5 days; death is result of peripheral and cranial nerve palsy lifespan of mutants is 45.5 days; death is result of peripheral and cranial nerve palsy   (MGI Ref ID J:108359)
    • the average lifespan of homozygotes in this study is 37 days   (MGI Ref ID J:126892)
    • median survival is 51 days   (MGI Ref ID J:170081)
  • homeostasis/metabolism phenotype
  • abnormal enzyme/coenzyme activity
    • levels of Ptgds2 activity are 3-fold higher in the cerebrum and 5-fold higher in the cerebellum of mutants compared to wild-type   (MGI Ref ID J:108359)
  • abnormal lipid level
    • galactosylsphingosine accumulation, with 14 fold higher levels than in wild-type, in the long bones   (MGI Ref ID J:165361)
    • abnormal sphingomyelin level
      • increase in total sphingomyelin in femurs, and in most of the sphingomyelin and ceramide containing saturated-unsaturated fatty acids   (MGI Ref ID J:165361)
  • decreased circulating insulin-like growth factor I level
    • IGF-1 plasma levels are reduced by 37.6%   (MGI Ref ID J:165361)
  • behavior/neurological phenotype
  • abnormal gait
    • wobbly gait appears at approximately 28-30 days of age   (MGI Ref ID J:126892)
  • ataxia   (MGI Ref ID J:108359)
  • impaired righting response
    • mutants have poorer righting response compared to wild-type or Ptgds2/Galc double mutants   (MGI Ref ID J:108359)
  • paraparesis   (MGI Ref ID J:126892)
  • paresis
    • moribund mice develop spastic paresis   (MGI Ref ID J:170081)
  • tremors
    • mutants barely stagger with strong intentional tremor   (MGI Ref ID J:108359)
    • mean age of onset 21 days   (MGI Ref ID J:126892)
  • nervous system phenotype
  • abnormal astrocyte morphology
    • there are hypertrophied astrocytes with large soma and thick-branched processes in mutant brains   (MGI Ref ID J:108359)
    • increased GFAP staining astrocytes are found in the cerebellar granular layer and pons   (MGI Ref ID J:126892)
    • astrocytosis
      • white matter exhibits similar reactive astrocytosis as double Galctwi Csf1op mice   (MGI Ref ID J:170081)
  • abnormal brain morphology
    • lectin-positive macrophages are found in the cerebellum, pons, and medulla more severely on this genetic background than when outcrossed to CAST/EiJ   (MGI Ref ID J:126892)
    • abnormal forebrain morphology
      • levels of psychosine are increased in the forebrain while levels of free sphingosine are lower compared to wild-type mice   (MGI Ref ID J:170081)
    • abnormal hindbrain morphology
      • dramatically increased concentration of psychosine in the pons/medulla which is not impacted by genetic background   (MGI Ref ID J:126892)
    • increased oligodendrocyte progenitor number
      • mutants exhibit an increase in oligodendrocyte progenitor cell (OPC) population compared to wild-type   (MGI Ref ID J:170081)
  • abnormal microglial cell morphology
    • microglia in mutant brains have increased levels of Ptgds2 protein and have irregular thick processes in the region of demyelination   (MGI Ref ID J:108359)
  • abnormal oligodendrocyte morphology
    • oligodendrocyte progenitor cells become hypertrophic and have short and stout processes instead of well branched fine processes as in wild-type mice   (MGI Ref ID J:170081)
    • decreased oligodendrocyte number
      • mutants exhibit a moderate decrease in oligodendrocytes   (MGI Ref ID J:170081)
  • abnormal sciatic nerve morphology
    • sciatic nerves are swollen and demyelination and myelin debris is found in moribund homozygotes   (MGI Ref ID J:126892)
  • demyelination
    • 39 day-old mutant brains exhibit demyelination; demyelination appears to be restricted to the CNS   (MGI Ref ID J:108359)
    • extensive demyelination is found in the cerebellar white matter at the moribund stage on this genetic background   (MGI Ref ID J:126892)
    • myelin degeneration in the white matter, with a preferential loss of myelin in large diameter axons   (MGI Ref ID J:170081)
    • sciatic nerves are severely demyelinated   (MGI Ref ID J:170081)
    • however, development (wrapping) of myelin from P15 to P30 is not affected   (MGI Ref ID J:170081)
  • hematopoietic system phenotype
  • abnormal microglial cell morphology
    • microglia in mutant brains have increased levels of Ptgds2 protein and have irregular thick processes in the region of demyelination   (MGI Ref ID J:108359)
  • abnormal osteoclast physiology
    • enhanced osteoclast activity   (MGI Ref ID J:165361)
  • immune system phenotype
  • abnormal microglial cell morphology
    • microglia in mutant brains have increased levels of Ptgds2 protein and have irregular thick processes in the region of demyelination   (MGI Ref ID J:108359)
  • abnormal osteoclast physiology
    • enhanced osteoclast activity   (MGI Ref ID J:165361)
  • growth/size/body phenotype
  • decreased body weight
    • body weight is reduced starting at P20   (MGI Ref ID J:165361)
    • weight loss
      • mutants start to lose weight after P35   (MGI Ref ID J:170081)
  • limbs/digits/tail phenotype
  • abnormal femur morphology
    • femurs are smaller and weigh 27% less than wild-type femurs at 32-33 days of age   (MGI Ref ID J:165361)
  • skeleton phenotype
  • abnormal bone remodeling
    • reduction of bone deposition in the metaphyseal and epiphyseal region of femurs, but no differences in mineral apposition rate   (MGI Ref ID J:165361)
    • abnormal osteoclast physiology
      • enhanced osteoclast activity   (MGI Ref ID J:165361)
  • abnormal femur morphology
    • femurs are smaller and weigh 27% less than wild-type femurs at 32-33 days of age   (MGI Ref ID J:165361)
  • abnormal long bone metaphysis morphology
    • metaphyseal region of femurs show structural differences, with reduced trabecular bone mainly in the secondary spongiosa   (MGI Ref ID J:165361)
  • abnormal skeleton development
    • growth of long bones is retarded   (MGI Ref ID J:165361)
  • decreased bone mineral density
    • mice exhibit features of osteopenia   (MGI Ref ID J:165361)
  • decreased bone trabecula number
    • decrease in the number of trabeculae in femurs   (MGI Ref ID J:165361)
  • decreased compact bone thickness
    • decrease in cortical bone thickness in femurs   (MGI Ref ID J:165361)
  • muscle phenotype
  • muscle twitch
    • mutants develop fine twitching in the neck around P20   (MGI Ref ID J:170081)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Galctwi/Galctwi

        CE/J
  • nervous system phenotype
  • abnormal brain white matter morphology
    • demonstrated by light and electron microscopic investigations   (MGI Ref ID J:13867)
  • abnormal nervous system physiology
    • this mutation is the first spontaneous model for endoneurial edema and increased endoneurial fluid pressure   (MGI Ref ID J:7115)
    • CNS inflammation
      • mice show greater macrophage infiltration than Psaptm1Suz mice   (MGI Ref ID J:78223)
    • demyelination   (MGI Ref ID J:13867)
      • mice show more severe demyelination than do homozygous Psaptm1Suz mice   (MGI Ref ID J:78223)
  • abnormal spinal cord white matter morphology
    • demonstrated by light and electron microscopic investigations   (MGI Ref ID J:13867)
  • axon degeneration
    • some degeneration is evident by light and electron microscopic investigations   (MGI Ref ID J:13867)
  • peripheral nervous system degeneration
    • both motor and sensory nerves are involved   (MGI Ref ID J:13867)
  • immune system phenotype
  • CNS inflammation
    • mice show greater macrophage infiltration than Psaptm1Suz mice   (MGI Ref ID J:78223)
  • homeostasis/metabolism phenotype
  • abnormal lipid level
    • 45-day old mice have a much greater accumulation of galactosylceramide in kidney is greater than in 4-month old Psap-deficient mice   (MGI Ref ID J:78223)
    • at 40 days, brain levels of psychosine (galactosylsphingosine) are much higher than in wild-type controls or Psap-deficient mice (223 pmol/mg vs 60 or 32 pmol/mg)   (MGI Ref ID J:78223)
  • edema
    • endoneurial edema contributing to increased endoneurial fluid pressure   (MGI Ref ID J:7115)
  • behavior/neurological phenotype
  • hindlimb paralysis
    • evident by terminal stages of the disease   (MGI Ref ID J:13867)
  • impaired limb coordination
    • particularly the hindlimbs   (MGI Ref ID J:13867)
  • tremors
    • clinically evident between 15 and 20 days of age, beginning as a fine tremor   (MGI Ref ID J:13867)
    • involves the whole body   (MGI Ref ID J:13867)
  • growth/size/body phenotype
  • decreased body size
    • mutant mice are smaller than normal siblings especially noticeable between 15 and 20 days of age   (MGI Ref ID J:13867)

Galctwi/Galctwi

        involves: C57BL/6J * CE/J
  • mortality/aging
  • premature death
    • affected mice live only about 3 months   (MGI Ref ID J:6390)
  • behavior/neurological phenotype
  • tremors   (MGI Ref ID J:6390)
  • growth/size/body phenotype
  • cachexia   (MGI Ref ID J:6390)
  • hematopoietic system phenotype
  • abnormal macrophage morphology
    • multinucleated macrophages contain a variety of abnormal inclusions   (MGI Ref ID J:6390)
  • immune system phenotype
  • abnormal macrophage morphology
    • multinucleated macrophages contain a variety of abnormal inclusions   (MGI Ref ID J:6390)
  • muscle phenotype
  • progressive muscle weakness   (MGI Ref ID J:6390)
  • nervous system phenotype
  • abnormal myelin sheath morphology
    • degeneration in both central and peripheral nervous systems   (MGI Ref ID J:6390)

Galctwi/Galctwi

        involves: CE/J
  • nervous system phenotype
  • abnormal glial cell apoptosis
    • at 40 days of age 10% of oligodendrocytes stain TUNEL positive and there is a correspondence between TUNEL staining and stage of demyelination with the oligodendrocytes with cytoplasmic swelling or thick cellular processes still negative for TUNEL stain and oligodendrocytes with thin or shrunken processes and relatively small soma positive for TUNEL stain   (MGI Ref ID J:57215)
  • abnormal oligodendrocyte morphology
    • at 15 days of age the only difference found in the cerebral cortex and corpus callosum is rare cells with focal swelling of the cell soma, but by 20 days of age the oligodendrocytes, identified as expressing glutathione S-transferase pi, display irregular, often polygonal swelling of the cell soma, with or without unstained vacuoles, and with thicker processes and some focal swelling, and after 25 days of age the oligodendrocyte cellular processes and soma become progressively shrunken with only markedly shrunken soma, which lack identifiable processes   (MGI Ref ID J:57215)
    • decreased oligodendrocyte number
      • there is gradual decrease in oligodendrocyte number after 35 days of age and very few remain in the cortical layers at 45 days of age   (MGI Ref ID J:57215)
  • cellular phenotype
  • abnormal glial cell apoptosis
    • at 40 days of age 10% of oligodendrocytes stain TUNEL positive and there is a correspondence between TUNEL staining and stage of demyelination with the oligodendrocytes with cytoplasmic swelling or thick cellular processes still negative for TUNEL stain and oligodendrocytes with thin or shrunken processes and relatively small soma positive for TUNEL stain   (MGI Ref ID J:57215)
  • homeostasis/metabolism phenotype
  • abnormal protein level
    • 50 times the normal amount of galactosylceramide is found in the kidneys of homozygous mice at 42 days of age with less significant increases in liver and lung   (MGI Ref ID J:7311)
    • no abnormal increases of galactosylceramide or sulfatide are noted in the spinal cord or sciatic nerve at any age   (MGI Ref ID J:7311)
  • renal/urinary system phenotype
  • *normal* renal/urinary system phenotype
    • light microscopy reveals no abnormalities in kidneys of 5 to 45 day old homozygous mice   (MGI Ref ID J:7313)
    • abnormal kidney morphology
      • light microscopy reveals no abnormalities in kidneys of 5 to 45 day old homozygous mice   (MGI Ref ID J:7313)
      • ultrastructural analysis shows increasing amounts of epithelial cell inclusions as mutant mice age   (MGI Ref ID J:7313)
      • abnormal kidney development
        • cytoplasmic inclusions are found in the thin limbs of the loop of Henle in developing papillae of 5 day old mice   (MGI Ref ID J:7313)
      • abnormal kidney epithelium morphology
        • ultrastructural analysis reveals an increasing amount of cytoplasmic inclusions as mice age   (MGI Ref ID J:7313)
        • inclusions are restricted to the thin limbs of the loop of Henle but occasionally are seen in the lumen of thta structure   (MGI Ref ID J:7313)
    • abnormal kidney physiology
      • demonstrated by the occurrence of cytoplsmic inclusions in kidney tissue   (MGI Ref ID J:7311)

Galctwi/Galctwi

        involves: C57BL/6J * CAST/EiJ * CE/J
  • mortality/aging
  • premature death
    • average lifespan is 61.4 days on this mixed genetic background, longer than on the congenic C57BL/6J background, and there is much wider variation in the life span of individuals, which ranges from 45 to 77 days   (MGI Ref ID J:126892)
  • behavior/neurological phenotype
  • abnormal gait
    • wobbly gait does not develop until 30 days of age or beyond which is delayed compared with the congenic C57BL/6J background on which a wobbly gait appears at approximately 28-30 days of age   (MGI Ref ID J:126892)
  • paraparesis
    • the longer lived homozygotes display more severe wasting of the hindlimbs when at moribund conditions than do those on a C57BL/6J congenic background   (MGI Ref ID J:126892)
  • tremors
    • mean age of tremor onset is 24 days which is delayed when compared with homozygotes on a congenic C57BL/6J background, which have a mean age of onset of 21 days   (MGI Ref ID J:126892)
  • nervous system phenotype
  • abnormal astrocyte morphology
    • increased GFAP staining astrocytes are found in the cerebellar granular layer to a greater degree on this mixed genetic background than homozygotes on the congenic C57BL/6J background, and increased GFAP staining of astrocytes is found in the pons but to a lesser degree on this mixed genetic background than in homozygotes on the congenic C57BL/6J background   (MGI Ref ID J:126892)
  • abnormal brain morphology
    • lectin-positive macrophages are found in the cerebellum, pons, and medulla in the moribund stage, although to a lesser degree than in homozygotes on a C57BL/6J congenic background   (MGI Ref ID J:126892)
    • abnormal hindbrain morphology
      • on both this mixed genetic background and on the congenic C57BL/6J background the concentration of psychosine in the pons/medulla is dramatically higher than normal and the level of increase is not impacted by genetic background   (MGI Ref ID J:126892)
      • abnormal medulla oblongata morphology   (MGI Ref ID J:126892)
      • abnormal pons morphology   (MGI Ref ID J:126892)
  • abnormal sciatic nerve morphology
    • sciatic nerves from longer-lived homozygotes on this mixed genetic background show a greater degree of swelling than those on the congenic C57BL/6J background, loss of discrete fasicules, increased cellularity of lipid-filled cells, and demyelination   (MGI Ref ID J:126892)
  • axonal spheroids
    • found in the axonal plexus of the granular layer and the cerebellar white matter, but generally not seen in the moribund homozygotes on the C57BL/6J congenic background which die at an earlier age   (MGI Ref ID J:126892)
  • demyelination
    • focal areas of demyelination are found in the cerebellar white matter at the moribund stage, but this is less severe than the extensive demyelination of the cerebellar white matter found in homozygotes on a C57BL/6J congenic background at the moribund stage   (MGI Ref ID J:126892)

Galctwi/Galctwi

        involves: 129P2/OlaHsd * C57BL/6J * CE/J
  • mortality/aging
  • premature death
    • average lifespan 48 days of age with nearly all dead before 58 days of age   (MGI Ref ID J:63197)

The following phenotype relates to a compound genotype created using this strain.
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Galctwi/Galctwi Il6+/?

        involves: 129S2/SvPas * C57BL/6J * CE/J
  • mortality/aging
  • premature death
    • average lifespan is 39.29 days average lifespan is 39.29 days   (MGI Ref ID J:56656)
  • cardiovascular system phenotype
  • abnormal blood-brain barrier function
    • modest disruption of the blood-brain barrier detected by immunohistochemical detection of IgG or albumin   (MGI Ref ID J:56656)
    • lipopolysaccharide injection results in increased blood-brain barrier disruption and further shortens lifespan   (MGI Ref ID J:56656)
  • homeostasis/metabolism phenotype
  • abnormal tumor necrosis factor level
    • hindbrain has elevated TNF levels (9.38 pg/mg versus 6.54 pg/mg)   (MGI Ref ID J:56656)
  • immune system phenotype
  • abnormal tumor necrosis factor level
    • hindbrain has elevated TNF levels (9.38 pg/mg versus 6.54 pg/mg)   (MGI Ref ID J:56656)
  • nervous system phenotype
  • abnormal blood-brain barrier function
    • modest disruption of the blood-brain barrier detected by immunohistochemical detection of IgG or albumin   (MGI Ref ID J:56656)
    • lipopolysaccharide injection results in increased blood-brain barrier disruption and further shortens lifespan   (MGI Ref ID J:56656)
  • abnormal cerebellum white matter morphology
    • the deep cerebellar white matter has periodic acid-Schiff staining globoid cells and lectin-positive macrophages   (MGI Ref ID J:56656)
  • demyelination
    • most severe in the hindbrain/cervical spinal cord   (MGI Ref ID J:56656)
  • gliosis
    • severe gliosis in both gray and white matter regions   (MGI Ref ID J:56656)
  • behavior/neurological phenotype
  • tremors
    • the average onset of twitching is 25.85 days   (MGI Ref ID J:56656)
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Research Applications
This mouse can be used to support research in many areas including:

Galctwi related

Neurobiology Research
Myelination Defects
Tremor Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Galctwi
Allele Name twitcher
Allele Type Spontaneous
Common Name(s) galc-; twi;
Strain of OriginCE/J
Gene Symbol and Name Galc, galactosylceramidase
Chromosome 12
Gene Common Name(s) 2310068B06Rik; A930008M05Rik; AW212969; AW413532; Gacy; RIKEN cDNA 2310068B06 gene; RIKEN cDNA A930008M05 gene; expressed sequence AW212969; expressed sequence AW413532; galactocerebrosidase; twi; twitcher;
Molecular Note Sequence analysis comparisons of cDNA from livers of mice homozygous for this allele and +/+ mice showed a G to A transition at codon 339. Northern analysis showed absence of the most abundant mRNA of mouse galactocerebrosidase in mice homozygous for this allele. [MGI Ref ID J:31433]

Genotyping

Genotyping Information

Genotyping Protocols

Galctwi, Pyrosequencing


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

D'Eustachio P; Clarke V. 1993. Localization of the twitcher (twi) mutation on mouse chromosome 12. Mamm Genome 4(11):684-6. [PubMed: 8281019]  [MGI Ref ID J:15634]

Duchen LW; Eicher EM; Jacobs JM; Scaravilli F; Teixeira F. 1980. Hereditary leucodystrophy in the mouse: the new mutant twitcher. Brain 103(3):695-710. [PubMed: 7417782]  [MGI Ref ID J:6390]

Kobayashi T; Yamanaka T; Jacobs JM; Teixeira F; Suzuki K. 1980. The Twitcher mouse: an enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease). Brain Res 202(2):479-83. [PubMed: 7437911]  [MGI Ref ID J:6423]

LeVine SM; Brown DC. 1997. IL-6 and TNFalpha expression in brains of twitcher, quaking and normal mice. J Neuroimmunol 73(1-2):47-56. [PubMed: 9058758]  [MGI Ref ID J:40116]

Matsushima GK; Taniike M; Glimcher LH; Grusby MJ; Frelinger JA; Suzuki K; Ting JP. 1994. Absence of MHC class II molecules reduces CNS demyelination, microglial/macrophage infiltration, and twitching in murine globoid cell leukodystrophy. Cell 78(4):645-56. [PubMed: 8069913]  [MGI Ref ID J:19914]

Suzuki K; Suzuki K. 1995. The twitcher mouse: a model for Krabbe disease and for experimental therapies. Brain Pathol 5(3):249-58. [PubMed: 8520724]  [MGI Ref ID J:29140]

Taniike M; Mohri I; Eguchi N; Beuckmann CT; Suzuki K; Urade Y. 2002. Perineuronal oligodendrocytes protect against neuronal apoptosis through the production of lipocalin-type prostaglandin D synthase in a genetic demyelinating model. J Neurosci 22(12):4885-96. [PubMed: 12077186]  [MGI Ref ID J:77531]

Wu YP; McMahon E; Kraine MR; Tisch R; Meyers A; Frelinger J; Matsushima GK; Suzuki K. 2000. Distribution and characterization of GFP(+) donor hematogenous cells in Twitcher mice after bone marrow transplantation. Am J Pathol 156(6):1849-54. [PubMed: 10854208]  [MGI Ref ID J:62662]

Galctwi related

Biswas S; Biesiada H; Williams TD; LeVine SM. 2002. Delayed clinical and pathological signs in twitcher (globoid cell leukodystrophy) mice on a C57BL/6 x CAST/Ei background. Neurobiol Dis 10(3):344-57. [PubMed: 12270695]  [MGI Ref ID J:126892]

Cachon-Gonzalez MB; Wang SZ; Ziegler R; Cheng SH; Cox TM. 2014. Reversibility of neuropathology in Tay-Sachs-related diseases. Hum Mol Genet 23(3):730-48. [PubMed: 24057669]  [MGI Ref ID J:204890]

Cantuti Castelvetri L; Givogri MI; Hebert A; Smith B; Song Y; Kaminska A; Lopez-Rosas A; Morfini G; Pigino G; Sands M; Brady ST; Bongarzone ER. 2013. The sphingolipid psychosine inhibits fast axonal transport in Krabbe disease by activation of GSK3beta and deregulation of molecular motors. J Neurosci 33(24):10048-56. [PubMed: 23761900]  [MGI Ref ID J:199164]

Cho SK; Gao N; Pearce DA; Lehrman MA; Hofmann SL. 2005. Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease. Glycobiology 15(6):637-48. [PubMed: 15647513]  [MGI Ref ID J:112499]

Contreras MA; Haq E; Uto T; Singh I; Singh AK. 2008. Psychosine-induced alterations in peroxisomes of twitcher mouse liver. Arch Biochem Biophys 477(2):211-8. [PubMed: 18602885]  [MGI Ref ID J:141880]

Contreras MA; Ries WL; Shanmugarajan S; Arboleda G; Singh I; Singh AK. 2010. Factors that affect postnatal bone growth retardation in the twitcher murine model of Krabbe disease. Biochim Biophys Acta 1802(7-8):601-8. [PubMed: 20441793]  [MGI Ref ID J:165361]

Costantino-Ceccarini E; Luddi A; Volterrani M; Strazza M; Rafi MA ; Wenger DA. 1999. Transduction of cultured oligodendrocytes from normal and twitcher mice by a retroviral vector containing human galactocerebrosidase (GALC) cDNA. Neurochem Res 24(2):287-93. [PubMed: 9972877]  [MGI Ref ID J:52953]

Duchen LW. 1981. A new neurological mutant "twitcher" (twi) Mouse News Lett 61:47.  [MGI Ref ID J:13867]

Duchen LW; Eicher EM; Jacobs JM; Scaravilli F; Teixeira F. 1980. Hereditary leucodystrophy in the mouse: the new mutant twitcher. Brain 103(3):695-710. [PubMed: 7417782]  [MGI Ref ID J:6390]

Ezoe T; Vanier MT; Oya Y; Popko B; Tohyama J; Matsuda J; Suzuki K; Suzuki K. 2000. Biochemistry and neuropathology of mice doubly deficient in synthesis and degradation of galactosylceramide. J Neurosci Res 59(2):170-8. [PubMed: 10650875]  [MGI Ref ID J:113298]

Ezoe T; Vanier MT; Oya Y; Popko B; Tohyama J; Matsuda J; Suzuki K; Suzuki K. 2000. Twitcher mice with only a single active galactosylceramide synthase gene exhibit clearly detectable but therapeutically minor phenotypic improvements. J Neurosci Res 59(2):179-87. [PubMed: 10650876]  [MGI Ref ID J:113251]

Galbiati F; Basso V; Cantuti L; Givogri MI; Lopez-Rosas A; Perez N; Vasu C; Cao H; van Breemen R; Mondino A; Bongarzone ER. 2007. Autonomic denervation of lymphoid organs leads to epigenetic immune atrophy in a mouse model of Krabbe disease. J Neurosci 27(50):13730-8. [PubMed: 18077684]  [MGI Ref ID J:130564]

Haq E; Contreras MA; Giri S; Singh I; Singh AK. 2006. Dysfunction of peroxisomes in twitcher mice brain: a possible mechanism of psychosine-induced disease. Biochem Biophys Res Commun 343(1):229-38. [PubMed: 16530726]  [MGI Ref ID J:107482]

Hawkins-Salsbury JA; Qin EY; Reddy AS; Vogler CA; Sands MS. 2012. Oxidative stress as a therapeutic target in globoid cell leukodystrophy. Exp Neurol 237(2):444-52. [PubMed: 22849820]  [MGI Ref ID J:193356]

Higashi Y; Komiyama A; Suzuki K. 1992. The twitcher mouse: immunocytochemical study of Ia expression in macrophages. J Neuropathol Exp Neurol 51(1):47-57. [PubMed: 1740674]  [MGI Ref ID J:2124]

Huppes W; De Groot CJ; Ostendorf RH; Bauman JG; Gossen JA; Smit V; Vijg J; Dijkstra CD. 1992. Detection of migrated allogeneic oligodendrocytes throughout the central nervous system of the galactocerebrosidase-deficient twitcher mouse. J Neurocytol 21(2):129-36. [PubMed: 1348528]  [MGI Ref ID J:1327]

Igisu H; Suzuki K. 1984. Glycolipids of the spinal cord, sciatic nerve, and systemic organs of the twitcher mouse. J Neuropathol Exp Neurol 43(1):22-36. [PubMed: 6693925]  [MGI Ref ID J:7311]

Igisu H; Suzuki K. 1984. Progressive accumulation of toxic metabolite in a genetic leukodystrophy. Science 224(4650):753-5. [PubMed: 6719111]  [MGI Ref ID J:7427]

Ijichi K; Brown GD; Moore CS; Lee JP; Winokur PN; Pagarigan R; Snyder EY; Bongarzone ER; Crocker SJ. 2013. MMP-3 mediates psychosine-induced globoid cell formation: Implications for leukodystrophy pathology. Glia 61(5):765-77. [PubMed: 23404611]  [MGI Ref ID J:194963]

Kobayashi T; Yamanaka T; Jacobs JM; Teixeira F; Suzuki K. 1980. The Twitcher mouse: an enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease). Brain Res 202(2):479-83. [PubMed: 7437911]  [MGI Ref ID J:6423]

Komiyama A; Suzuki K. 1994. Progressive dysfunction of twitcher Schwann cells is evaluated better in vitro than in vivo. Brain Res 637(1-2):106-13. [PubMed: 8180787]  [MGI Ref ID J:17678]

Komiyama A; Suzuki K. 1992. Progressive impairment of Schwann cell proliferation in vitro in murine globoid cell leukodystrophy (twitcher). Brain Res 598(1-2):1-9. [PubMed: 1486471]  [MGI Ref ID J:3584]

Kondo Y; Adams JM; Vanier MT; Duncan ID. 2011. Macrophages counteract demyelination in a mouse model of globoid cell leukodystrophy. J Neurosci 31(10):3610-24. [PubMed: 21389217]  [MGI Ref ID J:170081]

LeVine SM; Brown DC. 1997. IL-6 and TNFalpha expression in brains of twitcher, quaking and normal mice. J Neuroimmunol 73(1-2):47-56. [PubMed: 9058758]  [MGI Ref ID J:40116]

LeVine SM; Torres MV. 1992. Morphological features of degenerating oligodendrocytes in twitcher mice. Brain Res 587(2):348-52. [PubMed: 1525668]  [MGI Ref ID J:2125]

LeVine SM; Wetzel DL; Eilert AJ. 1994. Neuropathology of twitcher mice: examination by histochemistry, immunohistochemistry, lectin histochemistry and Fourier transform infrared microspectroscopy. Int J Dev Neurosci 12(4):275-88. [PubMed: 7526605]  [MGI Ref ID J:22147]

Luddi A; Strazza M; Carbone M; Moretti E; Costantino-Ceccarini E. 2005. Galactosylceramidase deficiency causes sperm abnormalities in the mouse model of globoid cell leukodystrophy. Exp Cell Res 304(1):59-68. [PubMed: 15707574]  [MGI Ref ID J:98178]

Luzi P; Abraham RM; Rafi MA; Curtis M; Hooper DC; Wenger DA. 2009. Effects of treatments on inflammatory and apoptotic markers in the CNS of mice with globoid cell leukodystrophy. Brain Res 1300:146-58. [PubMed: 19748497]  [MGI Ref ID J:158553]

Luzi P; Rafi MA; Zaka M; Curtis M; Vanier MT; Wenger DA. 2001. Generation of a mouse with low galactocerebrosidase activity by gene targeting: a new model of globoid cell leukodystrophy (Krabbe disease). Mol Genet Metab 73(3):211-23. [PubMed: 11461188]  [MGI Ref ID J:78691]

Matsuda J; Vanier MT; Saito Y; Tohyama J; Suzuki K; Suzuki K. 2001. A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse. Hum Mol Genet 10(11):1191-9. [PubMed: 11371512]  [MGI Ref ID J:78223]

Matsushima GK; Taniike M; Glimcher LH; Grusby MJ; Frelinger JA; Suzuki K; Ting JP. 1994. Absence of MHC class II molecules reduces CNS demyelination, microglial/macrophage infiltration, and twitching in murine globoid cell leukodystrophy. Cell 78(4):645-56. [PubMed: 8069913]  [MGI Ref ID J:19914]

Meng XL; Shen JS; Kawagoe S; Ohashi T; Brady RO; Eto Y. 2010. Induced pluripotent stem cells derived from mouse models of lysosomal storage disorders. Proc Natl Acad Sci U S A 107(17):7886-91. [PubMed: 20385825]  [MGI Ref ID J:159373]

Mohri I; Taniike M; Taniguchi H; Kanekiyo T; Aritake K; Inui T; Fukumoto N; Eguchi N; Kushi A; Sasai H; Kanaoka Y; Ozono K; Narumiya S; Suzuki K; Urade Y. 2006. Prostaglandin D2-mediated microglia/astrocyte interaction enhances astrogliosis and demyelination in twitcher. J Neurosci 26(16):4383-93. [PubMed: 16624958]  [MGI Ref ID J:108359]

Neri M; Ricca A; di Girolamo I; Alcala'-Franco B; Cavazzin C; Orlacchio A; Martino S; Naldini L; Gritti A. 2011. Neural stem cell gene therapy ameliorates pathology and function in a mouse model of globoid cell leukodystrophy. Stem Cells 29(10):1559-71. [PubMed: 21809420]  [MGI Ref ID J:190205]

Ohno M; Komiyama A; Martin PM; Suzuki K. 1993. MHC class II antigen expression and T-cell infiltration in the demyelinating CNS and PNS of the twitcher mouse. Brain Res 625(2):186-96. [PubMed: 8275302]  [MGI Ref ID J:15111]

Ohno M; Komiyama A; Martin PM; Suzuki K. 1993. Proliferation of microglia/macrophages in the demyelinating CNS and PNS of twitcher mouse. Brain Res 602(2):268-74. [PubMed: 8448672]  [MGI Ref ID J:3948]

Ono J; Harada K; Takahashi M; Maeda M; Ikenaka K; Sakurai K; Sakai N; Kagawa T; Fritz-Zieroth B; Nagai T; Nihei A; Hashimoto S; Okada S. 1995. Differentiation between dysmyelination and demyelination using magnetic resonance diffusional anisotropy. Brain Res 671(1):141-8. [PubMed: 7728526]  [MGI Ref ID J:22996]

Pannuzzo G; Cardile V; Costantino-Ceccarini E; Alvares E; Mazzone D; Perciavalle V. 2010. A galactose-free diet enriched in soy isoflavones and antioxidants results in delayed onset of symptoms of Krabbe disease in twitcher mice. Mol Genet Metab 100(3):234-40. [PubMed: 20418135]  [MGI Ref ID J:162440]

Pedchenko TV; LeVine SM. 1999. IL-6 deficiency causes enhanced pathology in Twitcher (globoid cell leukodystrophy) mice. Exp Neurol 158(2):459-68. [PubMed: 10415153]  [MGI Ref ID J:56656]

Pellegatta S; Tunici P; Poliani PL; Dolcetta D; Cajola L; Colombelli C; Ciusani E; Di Donato S; Finocchiaro G. 2006. The therapeutic potential of neural stem/progenitor cells in murine globoid cell leukodystrophy is conditioned by macrophage/microglia activation. Neurobiol Dis 21(2):314-23. [PubMed: 16199167]  [MGI Ref ID J:105739]

Powell HC; Knobler RL; Myers RR. 1983. Peripheral neuropathy in the Twitcher mutant. A new experimental model of endoneurial edema. Lab Invest 49(1):19-25. [PubMed: 6306338]  [MGI Ref ID J:7115]

Qin EY; Hawkins-Salsbury JA; Jiang X; Reddy AS; Farber NB; Ory DS; Sands MS. 2012. Bone marrow transplantation increases efficacy of central nervous system-directed enzyme replacement therapy in the murine model of globoid cell leukodystrophy. Mol Genet Metab 107(1-2):186-96. [PubMed: 22704480]  [MGI Ref ID J:188059]

Reddy AS; Patel JR; Vogler C; Klein RS; Sands MS. 2013. Central nervous system pathology progresses independently of KC and CXCR2 in globoid-cell leukodystrophy. PLoS One 8(6):e64647. [PubMed: 23755134]  [MGI Ref ID J:204256]

Sakai N; Inui K; Tatsumi N; Fukushima H; Nishigaki T; Taniike M; Nishimoto J; Tsukamoto H; Yanagihara I; Ozono K; Okada S. 1996. Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease. J Neurochem 66(3):1118-24. [PubMed: 8769874]  [MGI Ref ID J:31433]

Santambrogio S; Ricca A; Maderna C; Ieraci A; Aureli M; Sonnino S; Kulik W; Aimar P; Bonfanti L; Martino S; Gritti A. 2012. The galactocerebrosidase enzyme contributes to maintain a functional neurogenic niche during early post-natal CNS development. Hum Mol Genet 21(21):4732-50. [PubMed: 22859505]  [MGI Ref ID J:187995]

Scaravilli F; Jacobs JM. 1982. Improved myelination in nerve grafts from the leucodystrophic twitcher into trembler mice: evidence for enzyme replacement. Brain Res 237(1):163-72. [PubMed: 7074355]  [MGI Ref ID J:6749]

Scaravilli F; Jacobs JM. 1981. Peripheral nerve grafts in hereditary leukodystrophic mutant mice (twitcher). Nature 290(5801):56-8. [PubMed: 7207584]  [MGI Ref ID J:6477]

Scaravilli F; Suzuki K. 1983. Enzyme replacement in grafted nerve of twitcher mouse. Nature 305(5936):713-5. [PubMed: 6633639]  [MGI Ref ID J:7223]

Shen JS; Watabe K; Ohashi T; Eto Y. 2001. Intraventricular administration of recombinant adenovirus to neonatal twitcher mouse leads to clinicopathological improvements. Gene Ther 8(14):1081-7. [PubMed: 11526455]  [MGI Ref ID J:123201]

Skiba MC; Lyerla TA; Konola JT; Raghavan S. 1990. Somatic cell genetic analysis of the galactocerebrosidase gene: lack of complementation in human Krabbe disease/twitcher mouse cell hybrids. J Neurosci Res 27(4):472-8. [PubMed: 2079710]  [MGI Ref ID J:35073]

Smith BR; Santos MB; Marshall MS; Cantuti-Castelvetri L; Lopez-Rosas A; Li G; van Breemen R; Claycomb KI; Gallea JI; S Celej M; Crocker SJ; Givogri MI; Bongarzone ER. 2014. Neuronal inclusions of alpha-synuclein contribute to the pathogenesis of Krabbe disease. J Pathol 232(5):509-21. [PubMed: 24415155]  [MGI Ref ID J:208051]

Suzuki K; Suzuki K. 1995. The twitcher mouse: a model for Krabbe disease and for experimental therapies. Brain Pathol 5(3):249-58. [PubMed: 8520724]  [MGI Ref ID J:29140]

Suzuki K; Taniike M. 1995. Murine model of genetic demyelinating disease: the twitcher mouse. Microsc Res Tech 32(3):204-14. [PubMed: 8527855]  [MGI Ref ID J:29582]

Sweet HO; Davisson MT. 1995. Remutations at The Jackson Laboratory (Update to Mouse Genome 1993; 91:862-5 - J16313) Mouse Genome 93(4):1030-4.  [MGI Ref ID J:30778]

Takahashi H; Igisu H; Suzuki K; Suzuki K. 1984. Murine globoid cell leukodystrophy: the twitcher mouse. An ultrastructural study of the kidney. Lab Invest 50(1):42-50. [PubMed: 6319815]  [MGI Ref ID J:7313]

Taniike M; Mohri I; Eguchi N; Beuckmann CT; Suzuki K; Urade Y. 2002. Perineuronal oligodendrocytes protect against neuronal apoptosis through the production of lipocalin-type prostaglandin D synthase in a genetic demyelinating model. J Neurosci 22(12):4885-96. [PubMed: 12077186]  [MGI Ref ID J:77531]

Taniike M; Mohri I; Eguchi N; Irikura D; Urade Y; Okada S; Suzuki K. 1999. An apoptotic depletion of oligodendrocytes in the twitcher, a murine model of globoid cell leukodystrophy. J Neuropathol Exp Neurol 58(6):644-53. [PubMed: 10374755]  [MGI Ref ID J:57215]

Taniike M; Suzuki K. 1995. Proliferative capacity of oligodendrocytes in the demyelinating twitcher spinal cord. J Neurosci Res 40(3):325-32. [PubMed: 7745626]  [MGI Ref ID J:23073]

Taniike M; Suzuki K. 1994. Spacio-temporal progression of demyelination in twitcher mouse: with clinico-pathological correlation. Acta Neuropathol (Berl) 88(3):228-36. [PubMed: 7528964]  [MGI Ref ID J:20501]

Taylor RM; Lee JP; Palacino JJ; Bower KA; Li J; Vanier MT; Wenger DA; Sidman RL; Snyder EY. 2006. Intrinsic resistance of neural stem cells to toxic metabolites may make them well suited for cell non-autonomous disorders: evidence from a mouse model of Krabbe leukodystrophy. J Neurochem 97(6):1585-99. [PubMed: 16805770]  [MGI Ref ID J:119020]

Teixeira CA; Miranda CO; Sousa VF; Santos TE; Malheiro AR; Solomon M; Maegawa GH; Brites P; Sousa MM. 2014. Early axonal loss accompanied by impaired endocytosis, abnormal axonal transport, and decreased microtubule stability occur in the model of Krabbe's disease. Neurobiol Dis 66:92-103. [PubMed: 24607884]  [MGI Ref ID J:212010]

Tohyama J; Vanier MT; Suzuki K; Ezoe T; Matsuda J. 2000. Paradoxical influence of acid beta-galactosidase gene dosage on phenotype of the twitcher mouse (genetic galactosylceramidase deficiency) Hum Mol Genet 9(11):1699-707. [PubMed: 10861297]  [MGI Ref ID J:63197]

Visigalli I; Ungari S; Martino S; Park H; Cesani M; Gentner B; Sergi Sergi L; Orlacchio A; Naldini L; Biffi A. 2010. The galactocerebrosidase enzyme contributes to the maintenance of a functional hematopoietic stem cell niche. Blood 116(11):1857-66. [PubMed: 20511539]  [MGI Ref ID J:164533]

Vitner EB; Salomon R; Farfel-Becker T; Meshcheriakova A; Ali M; Klein AD; Platt FM; Cox TM; Futerman AH. 2014. RIPK3 as a potential therapeutic target for Gaucher's disease. Nat Med 20(2):204-8. [PubMed: 24441827]  [MGI Ref ID J:206864]

White AB; Givogri MI; Lopez-Rosas A; Cao H; van Breemen R; Thinakaran G; Bongarzone ER. 2009. Psychosine accumulates in membrane microdomains in the brain of krabbe patients, disrupting the raft architecture. J Neurosci 29(19):6068-77. [PubMed: 19439584]  [MGI Ref ID J:148760]

Whitfield PD; Sharp PC; Taylor R; Meikle P. 2001. Quantification of galactosylsphingosine in the twitcher mouse using electrospray ionization-tandem mass spectrometry. J Lipid Res 42(12):2092-5. [PubMed: 11734583]  [MGI Ref ID J:73367]

Wu YP; McMahon E; Kraine MR; Tisch R; Meyers A; Frelinger J; Matsushima GK; Suzuki K. 2000. Distribution and characterization of GFP(+) donor hematogenous cells in Twitcher mice after bone marrow transplantation. Am J Pathol 156(6):1849-54. [PubMed: 10854208]  [MGI Ref ID J:62662]

Yagi T; Matsuda J; Takikita S; Mohri I; Suzuki K; Suzuki K. 2004. Comparative clinico-pathological study of saposin-A-deficient (SAP-A-/-) and Twitcher mice. J Neuropathol Exp Neurol 63(7):721-34. [PubMed: 15290897]  [MGI Ref ID J:104960]

Yagi T; McMahon EJ; Takikita S; Mohri I; Matsushima GK; Suzuki K. 2004. Fate of donor hematopoietic cells in demyelinating mutant mouse, twitcher, following transplantation of GFP+ bone marrow cells. Neurobiol Dis 16(1):98-109. [PubMed: 15207267]  [MGI Ref ID J:91240]

Yeager AM; Brennan S; Tiffany C; Moser HW; Santos GW. 1984. Prolonged survival and remyelination after hematopoietic cell transplantation in the twitcher mouse. Science 225(4666):1052-4. [PubMed: 6382609]  [MGI Ref ID J:7576]

Yeager AM; Shinn C; Shinohara M; Pardoll DM. 1993. Hematopoietic cell transplantation in the twitcher mouse. The effects of pretransplant conditioning with graded doses of busulfan. Transplantation 56(1):185-90. [PubMed: 8101401]  [MGI Ref ID J:14381]

Zhou D; Cantu C rd; Sagiv Y; Schrantz N; Kulkarni AB; Qi X; Mahuran DJ; Morales CR; Grabowski GA; Benlagha K; Savage P; Bendelac A; Teyton L. 2004. Editing of CD1d-bound lipid antigens by endosomal lipid transfer proteins. Science 303(5657):523-7. [PubMed: 14684827]  [MGI Ref ID J:90443]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3300.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Frozen Products

Price (US dollars $)
Frozen Embryo $1650.00

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • Cryorecovery of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 10 and 14 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is no guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain, tel: 1-800-422-6423 or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $4290.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Frozen Products

Price (US dollars $)
Frozen Embryo $2145.00

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • Cryorecovery of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 10 and 14 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is no guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain, tel: 1-800-422-6423 or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   Untyped from the colony
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


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The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

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MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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