Strain Name:

BNT/LeJ

Stock Number:

000250

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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

Former Names Bent Tail    (Changed: 15-DEC-04 )
Type Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Type Inbred Strain;
Additional information on Inbred Strains.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
GenerationF79p
Generation Definitions

Appearance
black
Related Genotype: a/a

Important Note
This strain is segregating for Zic3Bn.

Description
In the early 1950s, a wild type female from the Namru strain was crossed with a bald hrba/hrba male and one of the pups produced was a male with a bent tail. Pedigree tests revealed the underlying mutation to be semidominant and carried on the X chromosome. The predominant phenotypic trait of kinked, shortened tails results from mis-formed, smaller, and absent tail vertebrae. Heterozygous females have varied expressivity spanning a broad phenotypic range including mice with no apparent phenotype and mice with multiply kinked and shortened tails. Hemizygous males have the highest expressivity; the tail is shortened in some cases to half the normal length and in the most severe cases the kinks in the tail will cause the tail to bend sharply. In males the tail kinks are more common in the distal than the proximal half of the tail. Heterozygous females are fertile, but hemizygous males and homozyous females have decreased viability and fertility. The number of affected offspring is lower than expected. This is believed to result from in utero death as evidenced by an increase in resorption. The kinked and shortened tails likely result from neural tube defects. More than 10% of BNT/Le embryos have exencephaly. Orofacial schisis, eye defects, abnormal transition from a lordotic to a kyphotic curvature during neurulation, and omphalocele have also been found in these embryos. Mutant mice are smaller in overall body size and have an increased incidence of an interfrontal bone. As with ZIC3 mutations in humans, situs ambiguus was found in more than 50% of Bn/Y males and 38% of carrier females. (Garber ED, 1952, Science; Garber ED, 1952, PNAS; Gruneberg H., 1955; Johnson DR, 1976; Gebbia et al., 1997; Klootwijk et al., 2000.)

Non-disjunction was found to occur in the BNT/Le-a/a Bn/+ strain at a frequency of at least 4.4% and is thought to be a characteristic of this inbred background rather than due to the bent tail mutation. When female heterozygotes were crossed to Mus castaneus no bent tail phenotype was found in more than 40 F1 female offspring examined, which the authors point to as an indication of the importance of modifier loci in the expression of this phenotype. (Carrel et al., 2000)

Development
The genetic origins of the BNT/Le inbred strain are partially uncertain. The bald allele of hairless (probably now extinct) arose in an albino strain of unknown origin (Garber ED, 1952 J. Heredity v. 43 p.45.). The Namru strain was being inbred from albino mice of ABC stock at the time that a female from this strain was bred to a homozygous bald (hrba/hrba) male (Garber and Hauth, 1950 J. Heredity v.41 p.122). One of the male pups born from this mating had the bent tail mutation and when mated to a wild type sibling, produced affected females (some very mildly) and wild type males. The strain currently held by The Jackson Laboratory derived from C57BL/6J congenic breeder pairs sent by A. B. Griffin in April, 1960. The records indicate that these mice were at least at N10 (possibly N12) and they were sibling mated when they arrived at The Jackson Laboratory until they reached F27 in July, 1968, at which time carrier males were outcrossed to B6CBAF1 females. The offspring were sibling mated after that and reached F83 in 1986. Embryos were frozen during 1985 and 1986. (Garber ED, 1952. PNAS; Garber ED, 1952. Science.)

Related Strains

Strains carrying other alleles of Zic3
023162   B6;129-Zic3tm2.1Jwb/J
View Strains carrying other alleles of Zic3     (1 strain)

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).
Heterotaxy, Visceral, 1, X-Linked; HTX1
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Vacterl Association, X-Linked, with or without Hydrocephalus; VACTERLX   (ZIC3)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Zic3Bn/Zic3+

        BNT/LeJ
  • growth/size/body phenotype
  • situs ambiguus
    • abnormal organ position, found in more than 50% of Bn/Y males and 38% of carrier females   (MGI Ref ID J:62608)
  • limbs/digits/tail phenotype
  • decreased caudal vertebrae number
    • fewer caudal vertebrae than normal   (MGI Ref ID J:63168)
  • kinked tail   (MGI Ref ID J:14983)
    • one or more bends   (MGI Ref ID J:13124)
  • short tail   (MGI Ref ID J:14983)
    • variable and milder severity compared to homozygous females and hemizygous males   (MGI Ref ID J:13124)
  • skeleton phenotype
  • decreased caudal vertebrae number
    • fewer caudal vertebrae than normal   (MGI Ref ID J:63168)

Zic3Bn/Zic3Bn

        BNT/LeJ
  • mortality/aging
  • partial prenatal lethality
    • approximately 1/4 of animals are lost due to early lethality   (MGI Ref ID J:63168)
  • premature death   (MGI Ref ID J:13124)
  • craniofacial phenotype
  • abnormal cranium morphology
    • interfrontal bone is often present and the skull is slightly wider than normal   (MGI Ref ID J:5776)
    • abnormal neurocranium morphology   (MGI Ref ID J:5776)
    • cranioschisis   (MGI Ref ID J:63168)
      • variable penetrance   (MGI Ref ID J:5776)
  • growth/size/body phenotype
  • postnatal growth retardation
    • in the most severely affected animals   (MGI Ref ID J:63168)
  • situs ambiguus
    • abnormal organ position, found in more than 50% of Bn/Y males and 38% of carrier females   (MGI Ref ID J:62608)
  • limbs/digits/tail phenotype
  • decreased caudal vertebrae number
    • fewer and smaller caudal vertebrae than normal; more severe than heterozygous female   (MGI Ref ID J:63168)
  • kinked tail   (MGI Ref ID J:13124)
  • short tail   (MGI Ref ID J:13124)
  • reproductive system phenotype
  • reduced fertility   (MGI Ref ID J:14983)
    • number of affected offspring is lower than expected   (MGI Ref ID J:13124)
  • skeleton phenotype
  • abnormal cranium morphology
    • interfrontal bone is often present and the skull is slightly wider than normal   (MGI Ref ID J:5776)
    • abnormal neurocranium morphology   (MGI Ref ID J:5776)
    • cranioschisis   (MGI Ref ID J:63168)
      • variable penetrance   (MGI Ref ID J:5776)
  • decreased caudal vertebrae number
    • fewer and smaller caudal vertebrae than normal; more severe than heterozygous female   (MGI Ref ID J:63168)
  • nervous system phenotype
  • exencephaly   (MGI Ref ID J:63168)
    • variable penetrance   (MGI Ref ID J:5776)
  • open neural tube   (MGI Ref ID J:63168)
    • variable penetrance, in the sacral region; omphalocele and ventral midline closure defects noted in small numbers of embryos   (MGI Ref ID J:5776)
  • embryogenesis phenotype
  • open neural tube   (MGI Ref ID J:63168)
    • variable penetrance, in the sacral region; omphalocele and ventral midline closure defects noted in small numbers of embryos   (MGI Ref ID J:5776)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Reproductive Biology Research
Meiotic Nondisjunction Studies

Research Tools
Genetics Research
      Meiotic Nondisjunction Studies

Zic3Bn related

Cell Biology Research
Transcriptional Regulation

Developmental Biology Research
Embryonic Lethality (Homozygous)
Eye Defects
Neural Tube Defects
Skeletal Defects

Neurobiology Research
Neural Tube Defects
Neurodevelopmental Defects
      Joubert syndrome

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Zic3Bn
Allele Name bent tail
Allele Type Spontaneous
Common Name(s) Bn;
Strain of Origin(NAMRU x Hr)F1
Gene Symbol and Name Zic3, zinc finger protein of the cerebellum 3
Chromosome X
Gene Common Name(s) Bn; HTX; HTX1; Ka; VACTERLX; ZNF203; bent tail; katun;
Molecular Note The Bn mutation is caused by a deletion of ~60 to 170 kb in size. The deletion is known to include Zic3 and DXMit208. [MGI Ref ID J:62608] [MGI Ref ID J:63168]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Aruga J; Nagai T; Tokuyama T; Hayashizaki Y; Okazaki Y; Chapman VM; Mikoshiba K. 1996. The mouse zic gene family. Homologues of the Drosophila pair-rule gene odd-paired. J Biol Chem 271(2):1043-7. [PubMed: 8557628]  [MGI Ref ID J:30607]

Gebbia M; Ferrero GB; Pilia G; Bassi MT; Aylsworth A; Penman-Splitt M; Bird LM; Bamforth JS; Burn J; Schlessinger D; Nelson DL; Casey B. 1997. X-linked situs abnormalities result from mutations in ZIC3 [see comments] Nat Genet 17(3):305-8. [PubMed: 9354794]  [MGI Ref ID J:59280]

Johnson DR. 1976. The interfrontal bone and mutant genes in the mouse. J Anat 121(3):507-13. [PubMed: 1018005]  [MGI Ref ID J:5776]

Klootwijk R; Franke B; van Der Zee CE; de Boer RT; Wilms W; Hol FA; Mariman EC. 2000. A deletion encompassing zic3 in bent tail, a mouse model for X-linked neural tube defects Hum Mol Genet 9(11):1615-22. [PubMed: 10861288]  [MGI Ref ID J:63168]

Nagai T; Aruga J; Takada S; Gunther T; Sporle R; Schughart K ; Mikoshiba K. 1997. The expression of the mouse Zic1, Zic2, and Zic3 gene suggests an essential role for Zic genes in body pattern formation. Dev Biol 182(2):299-313. [PubMed: 9070329]  [MGI Ref ID J:38554]

Zic3Bn related

Aruga J; Ogura H; Shutoh F; Ogawa M; Franke B; Nagao S; Mikoshiba K. 2004. Locomotor and oculomotor impairment associated with cerebellar dysgenesis in Zic3-deficient (Bent tail) mutant mice. Eur J Neurosci 20(8):2159-67. [PubMed: 15450095]  [MGI Ref ID J:101328]

Carrel T; Purandare SM; Harrison W; Elder F; Fox T; Casey B; Herman GE. 2000. The X-linked mouse mutation Bent tail is associated with a deletion of the Zic3 locus Hum Mol Genet 9(13):1937-42. [PubMed: 10942421]  [MGI Ref ID J:62608]

Franke B; Klootwijk R; Lemmers B; de Kovel CG; Steegers-Theunissen RP; Mariman EC. 2003. Phenotype of the neural tube defect mouse model bent tail is not sensitive to maternal folinic acid, myo-inositol, or zinc supplementation. Birth Defects Res Part A Clin Mol Teratol 67(12):979-84. [PubMed: 14745918]  [MGI Ref ID J:87145]

Garber ED. 1952. 'Bent-Tail,' A Dominant, Sex-Linked Mutation in the Mouse. Proc Natl Acad Sci U S A 38(10):876-9. [PubMed: 16589192]  [MGI Ref ID J:13124]

Gruneberg H. 1955. Genetical studies on the skeleton of the mouse. XVII. Bent-tail J Genet 53:551-62.  [MGI Ref ID J:14983]

Inoue T; Ogawa M; Mikoshiba K; Aruga J. 2008. Zic deficiency in the cortical marginal zone and meninges results in cortical lamination defects resembling those in type II lissencephaly. J Neurosci 28(18):4712-25. [PubMed: 18448648]  [MGI Ref ID J:134970]

Inoue T; Ota M; Mikoshiba K; Aruga J. 2007. Zic2 and Zic3 synergistically control neurulation and segmentation of paraxial mesoderm in mouse embryo. Dev Biol 306(2):669-84. [PubMed: 17490632]  [MGI Ref ID J:122556]

Inoue T; Ota M; Ogawa M; Mikoshiba K; Aruga J. 2007. Zic1 and Zic3 regulate medial forebrain development through expansion of neuronal progenitors. J Neurosci 27(20):5461-73. [PubMed: 17507568]  [MGI Ref ID J:121744]

Johnson DR. 1976. The interfrontal bone and mutant genes in the mouse. J Anat 121(3):507-13. [PubMed: 1018005]  [MGI Ref ID J:5776]

Klootwijk R; Franke B; van Der Zee CE; de Boer RT; Wilms W; Hol FA; Mariman EC. 2000. A deletion encompassing zic3 in bent tail, a mouse model for X-linked neural tube defects Hum Mol Genet 9(11):1615-22. [PubMed: 10861288]  [MGI Ref ID J:63168]

Klootwijk R; Schijvenaars MM; Mariman EC; Franke B. 2004. Further characterization of the genetic defect of the Bent tail mouse, a mouse model for human neural tube defects. Birth Defects Res A Clin Mol Teratol 70(11):880-4. [PubMed: 15526289]  [MGI Ref ID J:95076]

LYON MF; SEARLE AG; FORD CE; OHNO S. 1964. A MOUSE TRANSLOCATION SUPPRESSING SEX-LINKED VARIEGATION. Cytogenetics 15:306-23. [PubMed: 14248461]  [MGI Ref ID J:149655]

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.

Standard Supply

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

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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.

Standard Supply

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

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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.

Important Note

This strain is segregating for Zic3Bn.

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