Strain Name:

B6.Cg-Tg(Scgb1a1-Scnn1b)6608Bouc/J

Stock Number:

006438

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Cryopreserved - Ready for recovery

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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; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
 
Donating Investigator Wanda K. O'Neal,   Univ. of North Carolina at Chapel Hill

Description
These Scnn1b-transgenic mice overexpress the mouse nonvoltage-gated 1 beta, Scnn1b, under the direction of the rat secretoglobin, family 1A, member 1 (uteroglobin; Clara cell secretory protein) promoter. While the donating investigator reports that most hemizygous transgenic mice (80-90%) survive past postnatal day 14, hemizygous mice at The Jackson Laboratory exhibit an approximately 40% survival rate to weaning age. Hemizygous mice that do not survive die from airway obstruction asphyxia. Mice exhibit chronic inflammation with neutrophil infiltration, chronic mucus hypersecretion and emphysema. These Scnn1b-transgenic mice may be useful in studies of cystic fibrosis, and are available on different genetic backgrounds such as B6;C3H mixed (Stock No. 005315), B6C3Fe hybrid (Stock No. 006176), and C57BL6-congenic (Stock No. 006438).

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.

Importation of this model was supported by The Boomer Esiason Foundation.

Development
A transgenic construct containing the protein coding region of the mouse sodium channel, nonvoltage-gated 1 beta gene (Scnn1b) under the control of the rat secretoglobin, family 1A, member 1 (uteroglobin), Scgb1a1, promoter and SV40 polyadenylation site sequence was injected into fertilized B6C3F1 donor eggs. Founder line 6608 was established, and the strain subsequently was backcrossed to C57BL/6J for 12 generations.

Control Information

  Control
   Noncarrier
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Tg(Scgb1a1-Scnn1b)6608Bouc allele
005315   B6;C3H-Tg(Scgb1a1-Scnn1b)6608Bouc/J
006176   B6C3Fe-Tg(Scgb1a1-Scnn1b)6608Bouc/J
View Strains carrying   Tg(Scgb1a1-Scnn1b)6608Bouc     (2 strains)

View Strains carrying other alleles of Scgb1a1     (5 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.
Cystic Fibrosis; CF
- Potential model based on transgenic expression of a human gene that is associated with this disease. Phenotypic similarity to the human disease has not been tested.
Bronchiectasis with or without Elevated Sweat Chloride 1; BESC1   (SCNN1B)
Liddle Syndrome   (SCNN1B)
Pseudohypoaldosteronism, Type I, Autosomal Recessive; PHA1B   (SCNN1B)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

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

Tg(Scgb1a1-Scnn1b)6608Bouc/0

        involves: C3H * C57BL/6
  • mortality/aging
  • postnatal lethality
    • although transgenic animals are born at the expected Mendelian ratios, 50% of transgenic mice of both sexes die from the first days of postnatal life through 4 weeks of age   (MGI Ref ID J:91139)
  • respiratory system phenotype
  • abnormal respiratory mucosa goblet cell morphology
    • airway epithelia of adult transgenic mice exhibit goblet cell metaplasia   (MGI Ref ID J:91139)
  • abnormal respiratory system physiology
    • increased airway absorption of Na+ is demonstrated by elevation of basal and amiloride-sensitive short-circuit currents (Isc) across freshly excised adult and neonatal tracheal tissue of transgenic mice over those of nontransgenic littermates; in contrast, both forskolin and UTP fail to increase chloride channel activation in amiloride pretreated transgenic tracheae above wildtype levels   (MGI Ref ID J:91139)
    • abnormal mucociliary clearance
      • lung histology of all transgenic mice is normal at birth, but those that die early exhibit severe obstruction of small and large airways by mucus plaques and plugs; those euthanized after 4 weeks have less severe mucus obstruction with dilation of airspaces distal to blockages   (MGI Ref ID J:91139)
      • airway mucus of transgenic mice is significantly more concentrated than that of nontransgenic littermates; electron and light microscopy reveal adhesion of mucus to airway epithelia and reduction of periciliary liquid (PCL) height in tracheae and bronchi of transgenic mice   (MGI Ref ID J:91139)
      • the rate of clearance of a fluorescent dye demonstrates slower in vitro mucus transport in the lower airways of transgenic mice than of nontransgenic littermates, and whereas intratracheally instilled Haemophilus influenzae or Pseudomonas aeruginosa were almost completely cleared within 3 days from the lungs of wildtype mice, transgenic mice retained a significant bacterial burden   (MGI Ref ID J:91139)
    • lung inflammation
      • histopathologic examination of the lungs reveals evidence of chronic bronchitis, the lumina of conducting airways populated with macrophages and neutrophils; however, the submucosa contain few inflammatory cells   (MGI Ref ID J:91139)
      • bronchoalveolar lavage (BAL) fluid and lung homogenates of adult, but not neonatal, transgenic mice contain significantly higher levels of macrophage inflammatory protein 2 (MIP-2) and KC than do those of nontransgenic littermates   (MGI Ref ID J:91139)
    • respiratory distress
      • early deaths of transgenic mice result from asphyxia due to severe airway obstruction, evidenced by frequent observation of intercostal and subdiaphragmatic retractions   (MGI Ref ID J:91139)
  • immune system phenotype
  • lung inflammation
    • histopathologic examination of the lungs reveals evidence of chronic bronchitis, the lumina of conducting airways populated with macrophages and neutrophils; however, the submucosa contain few inflammatory cells   (MGI Ref ID J:91139)
    • bronchoalveolar lavage (BAL) fluid and lung homogenates of adult, but not neonatal, transgenic mice contain significantly higher levels of macrophage inflammatory protein 2 (MIP-2) and KC than do those of nontransgenic littermates   (MGI Ref ID J:91139)
View Research Applications

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

Developmental Biology Research
Internal/Organ Defects
      lung
Perinatal Lethality

Immunology, Inflammation and Autoimmunity Research
Cystic Fibrosis

Internal/Organ Research
Lung Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(Scgb1a1-Scnn1b)6608Bouc
Allele Name transgene insertion 6608, Richard Boucher
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) betaENaC-Tg;
Mutation Made By Wanda O'Neal,   Univ. of North Carolina at Chapel Hill
Strain of Origin(C3H x C57BL/6)F1
Expressed Gene Scnn1b, sodium channel, nonvoltage-gated 1 beta, mouse, laboratory
Promoter Scgb1a1, secretoglobin, family 1A, member 1 (uteroglobin), rat
General Note A second transgenic line, Tg(Scgb1a1-Scnn1b)6047Bouc, was created in the same genetic background. The phenotypes of the two lines are indistinguishable.

Complete histological examination of multiple organs and systems reveals no pathology of organs other than lungs of transgenic mice.

Molecular Note Approximately 2330 base pairs of 5' flanking sequence from the rat airway-specific Scgb1a1 (also called the Clara cell secretory protein) gene were inserted into the pTG1 vector upstream of exon 1 (104 base pairs, including the transcription initiation site), intron 1 (947 base pairs) and 10 base pairs of exon 2 from the mouse transthyretin gene; the protein-coding region of the mouse Scnn1b cDNA, inserted just downstream of the transthyretin exon 2 splice acceptor, is followed by the SV40 early-region polyadenylation signal. In situ hybridization with a transgene-specific antisense probe confirmed expression in airway epithelia of the lung. [MGI Ref ID J:101822] [MGI Ref ID J:101823] [MGI Ref ID J:91139]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

Tg(Scgb1a1-Scnn1b), High Resolution Melting
Tg(Scgb1a1-Scnn1b), Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Mall M; Grubb BR; Harkema JR; O'Neal WK; Boucher RC. 2004. Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice. Nat Med 10(5):487-93. [PubMed: 15077107]  [MGI Ref ID J:91139]

Additional References

Tg(Scgb1a1-Scnn1b)6608Bouc related

Grubb BR; O'Neal WK; Ostrowski LE; Kreda SM; Button B; Boucher RC. 2012. Transgenic hCFTR expression fails to correct beta-ENaC mouse lung disease. Am J Physiol Lung Cell Mol Physiol 302(2):L238-47. [PubMed: 22003093]  [MGI Ref ID J:183328]

Hackett BP; Gitlin JD. 1992. Cell-specific expression of a Clara cell secretory protein-human growth hormone gene in the bronchiolar epithelium of transgenic mice. Proc Natl Acad Sci U S A 89(19):9079-83. [PubMed: 1409605]  [MGI Ref ID J:101823]

Hector A; Kormann MS; Mack I; Latzin P; Casaulta C; Kieninger E; Zhou Z; Yildirim AO; Bohla A; Rieber N; Kappler M; Koller B; Eber E; Eickmeier O; Zielen S; Eickelberg O; Griese M; Mall MA; Hartl D. 2011. The chitinase-like protein YKL-40 modulates cystic fibrosis lung disease. PLoS One 6(9):e24399. [PubMed: 21949714]  [MGI Ref ID J:177680]

Johannesson B; Hirtz S; Schatterny J; Schultz C; Mall MA. 2012. CFTR regulates early pathogenesis of chronic obstructive lung disease in betaENaC-overexpressing mice. PLoS One 7(8):e44059. [PubMed: 22937152]  [MGI Ref ID J:191673]

Livraghi A; Grubb BR; Hudson EJ; Wilkinson KJ; Sheehan JK; Mall MA; O'Neal WK; Boucher RC; Randell SH. 2009. Airway and lung pathology due to mucosal surface dehydration in {beta}-epithelial Na+ channel-overexpressing mice: role of TNF-{alpha} and IL-4R{alpha} signaling, influence of neonatal development, and limited efficacy of glucocorticoid treatment. J Immunol 182(7):4357-67. [PubMed: 19299736]  [MGI Ref ID J:147194]

Livraghi-Butrico A; Kelly EJ; Wilkinson KJ; Rogers TD; Gilmore RC; Harkema JR; Randell SH; Boucher RC; O'Neal WK; Grubb BR. 2013. Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways. Am J Physiol Lung Cell Mol Physiol 304(7):L469-80. [PubMed: 23377346]  [MGI Ref ID J:195159]

Mall MA; Button B; Johannesson B; Zhou Z; Livraghi A; Caldwell RA; Schubert SC; Schultz C; O'Neal WK; Pradervand S; Hummler E; Rossier BC; Grubb BR; Boucher RC. 2010. Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice. J Biol Chem 285(35):26945-55. [PubMed: 20566636]  [MGI Ref ID J:166185]

Marcos V; Zhou Z; Yildirim AO; Bohla A; Hector A; Vitkov L; Wiedenbauer EM; Krautgartner WD; Stoiber W; Belohradsky BH; Rieber N; Kormann M; Koller B; Roscher A; Roos D; Griese M; Eickelberg O; Doring G; Mall MA; Hartl D. 2010. CXCR2 mediates NADPH oxidase-independent neutrophil extracellular trap formation in cystic fibrosis airway inflammation. Nat Med 16(9):1018-23. [PubMed: 20818377]  [MGI Ref ID J:164429]

Ostrowski LE; Hutchins JR; Zakel K; O'Neal WK. 2003. Targeting expression of a transgene to the airway surface epithelium using a ciliated cell-specific promoter. Mol Ther 8(4):637-45. [PubMed: 14529837]  [MGI Ref ID J:101822]

Health & husbandry

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.

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, these mice are bred as hemizygotes. While the donating investigator reports that most hemizygous transgenic mice (80-90%) survive past postnatal day 14, hemizygous mice at The Jackson Laboratory exhibit an approximately 40% survival rate to weaning age.

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* $2525.00
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* $3283.00
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.

Control Information

  Control
   Noncarrier
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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.
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"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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