Overview

This inbred mutant strain carries the bent tail mutation (Zic3^Bn).

Genetic overview

Genetic Background	Generation

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Research Applications

Research Tools
Reproductive Biology Research
Developmental Biology Research
Neurobiology Research
Cell Biology Research
Mouse/Human Gene Homologs

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Important Note

This strain is segregating for Zic3^Bn.

Detailed Description

In the early 1950s, a wild type female from the Namru strain was crossed with a bald hr^ba/hr^ba 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 (hr^ba/hr^ba) 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.)

Control Suggestions

Wild-type from the colony

Additional Information

Considerations for Choosing Controls

Genetics

Zic3^Bn

Allele Symbol: Zic3^Bn

Allele Name	bent tail
Allele Type	Spontaneous
Allele Synonym(s)	Bn
Gene Symbol and Name	Zic3, zinc finger protein of the cerebellum 3
Gene Synonym(s)
Strain of Origin	(NAMRU x Hr^ba)F1
Chromosome	X
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.

Disease/Phenotype

Disease Terms

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).

visceral heterotaxy

Research Areas By Phenotype

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

Research Tools
- Genetics Research
  - Meiotic Nondisjunction Studies
Reproductive Biology Research
- Meiotic Nondisjunction Studies

Genotype: Zic3^Bn related

Developmental Biology Research
- Embryonic Lethality (Homozygous)
- Eye Defects
- Neural Tube Defects
- Skeletal Defects
Neurobiology Research
- Neurodevelopmental Defects
  - Joubert syndrome
- Neural Tube Defects
Cell Biology Research
- Transcriptional Regulation
Mouse/Human Gene Homologs
- situs inversus

Mammalian Phenotype Terms by Genotype

The following phenotype relates to a compound genotype created using this strain

Genotype: Zic3^Bn/Zic3⁺
BNT/LeJ

growth/size/body region phenotype

situs ambiguus
- abnormal organ position, found in more than 50% of Bn/Y males and 38% of carrier females

limbs/digits/tail phenotype

decreased caudal vertebrae number
- fewer caudal vertebrae than normal

kinked tail
- one or more bends

short tail
- variable and milder severity compared to homozygous females and hemizygous males

skeleton phenotype

decreased caudal vertebrae number
- fewer caudal vertebrae than normal

Genotype: Zic3^Bn/Zic3^Bn
BNT/LeJ

craniofacial phenotype

abnormal interfrontal bone morphology
- interfrontal bone is often present

abnormal neurocranium morphology

cranioschisis
- variable penetrance

increased cranium width
- the skull is slightly wider than normal

limbs/digits/tail phenotype

decreased caudal vertebrae number
- fewer and smaller caudal vertebrae than normal; more severe than heterozygous female

kinked tail

short tail

mortality/aging

premature death

prenatal lethality, incomplete penetrance
- approximately 1/4 of animals are lost due to early lethality

nervous system phenotype

exencephaly
- variable penetrance

open neural tube
- variable penetrance, in the sacral region; omphalocele and ventral midline closure defects noted in small numbers of embryos

reproductive system phenotype

reduced fertility
- number of affected offspring is lower than expected

skeleton phenotype

abnormal interfrontal bone morphology
- interfrontal bone is often present

abnormal neurocranium morphology

cranioschisis
- variable penetrance

decreased caudal vertebrae number
- fewer and smaller caudal vertebrae than normal; more severe than heterozygous female

increased cranium width
- the skull is slightly wider than normal

embryo phenotype

open neural tube
- variable penetrance, in the sacral region; omphalocele and ventral midline closure defects noted in small numbers of embryos

growth/size/body region phenotype

postnatal growth retardation
- in the most severely affected animals

situs ambiguus
- abnormal organ position, found in more than 50% of Bn/Y males and 38% of carrier females

References

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):1043-7PubMed: 8557628 MGI: 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):305-8PubMed: 9354794 MGI: J:59280
Johnson DR. 1976. The interfrontal bone and mutant genes in the mouse. J Anat 121(3):507-13PubMed: 1018005 MGI: 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):1615-22PubMed: 10861288 MGI: 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):299-313PubMed: 9070329 MGI: J:38554

Additional - Zic3^Bn related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting
Breeding Considerations

The X-linked bent tail (Bn) mutation is segregating in this strain. The mating scheme is wild-type (for Bn) female x Bn/Y male or HET (for Bn) female x wild-type (for Bn) male. There is some sterility within this strain.
- Additional Breeding and Husbandry Support
Appearance
- black
  Related Genotype: a/a
Citation
When using the BNT/LeJ mouse strain in a publication, please include JAX stock #000250 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

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

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service/Product	Description	Price
	Heterozygous or Wild-type for Bn...one pair minimum

Payment Terms and Conditions

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 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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

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General Terms and Conditions

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Licensing Information

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Genetic overview

Research Applications

Base Price

Important Note

Detailed Description

Development

Control Suggestions

Additional Information

Zic3Bn

Allele Symbol: Zic3Bn

Disease Terms

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).

Research Areas By Phenotype

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

Genotype: Zic3Bn related

Mammalian Phenotype Terms by Genotype

Genotype: Zic3Bn/Zic3+BNT/LeJ

growth/size/body region phenotype

limbs/digits/tail phenotype

skeleton phenotype

Genotype: Zic3Bn/Zic3BnBNT/LeJ

craniofacial phenotype

limbs/digits/tail phenotype

mortality/aging

nervous system phenotype

reproductive system phenotype

skeleton phenotype

embryo phenotype

growth/size/body region phenotype

References

Additional References

Additional - Zic3Bn related

Genotyping Protocols

Breeding Considerations

Appearance

Citation

Animal Health Reports

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

Live Mouse

Cryorecovery - Pricing

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms Of Use

Licensing Information

JAX® Mice, Products & Services Conditions of Use

No Warranty

Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Zic3^Bn

Allele Symbol: Zic3^Bn

Genotype: Zic3^Bn related

Genotype: Zic3^Bn/Zic3⁺
BNT/LeJ

Genotype: Zic3^Bn/Zic3^Bn
BNT/LeJ

Additional - Zic3^Bn related