Go to JAX^® Mice Query Form

General Terms and Conditions	Additional Licensing and Use Restrictions

Genes & Alleles

Scgb1a1;   Scnn1b;   Tg(Scgb1a1-Scnn1b)6608Bouc;

---

Product Information

Strain Details

Type JAX® GEMM® Strain - Mutant Stock Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Transgenic Mating System See Colony Maintenance         (Female x Male) Species laboratory mouse Donating Investigator Wanda O'Neal,   Univ. of North Carolina at Chapel Hill Generation N?+N2pN1

Strain 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. Approximately 40-60% of hemizygous mice die between birth and 4 weeks of age due to airway obstruction asphyxia. Histological analysis reveals that mucus accumulation, plaques and plugs in airways occur postnatally. Basal and amiloride-sensitive short-circuit currents in tracheal tissue are increased. Airway surface liquid (ASL) volume, mucus transport and clearance are reduced. Bronchial lavage and histological analysis shows mutant mice exhibit characteristics of cystic fibrosis lung disease including chronic bronchitis, airway inflammation, airway lumen infiltration of macrophage and neutrophils, and goblet cell metaplasia. 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).

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

Strain 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.

Related Disease (OMIM) Terms Cystic Fibrosis; CF

Mammalian Phenotype Terms assigned by genotype Tg(Scgb1a1-Scnn1b)6608Bouc/0         involves: C3H * C57BL/6 respiratory system phenotype abnormal respiratory mucosa goblet cell morphology (MGI Ref ID J:91139) airway epithelia of adult transgenic mice exhibit goblet cell metaplasia abnormal respiratory system physiology (MGI Ref ID J:91139) 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 abnormal mucociliary clearance (MGI Ref ID J:91139) 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 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 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 lung inflammation (MGI Ref ID J:91139) 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 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 respiratory distress (MGI Ref ID J:91139) early deaths of transgenic mice result from asphyxia due to severe airway obstruction, evidenced by frequent observation of intercostal and subdiaphragmatic retractions lethality-postnatal postnatal lethality (MGI Ref ID J:91139) 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 immune system phenotype lung inflammation (MGI Ref ID J:91139) 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 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

Gene & Allele Details

Allele Symbol		Tg(Scgb1a1-Scnn1b)6608Bouc
Allele Name		transgene insertion 6608, Richard Boucher
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]

Control Information

	Control
	Noncarrier
	100010 B6C3F1/J
Considerations for Choosing Controls

Genotyping Protocols

Tg(Scgb1a1-Scnn1b)6608Bouc

Colony Maintenance

Breeding & Husbandry	When maintaining a live colony, hemizygous mice are bred to B6C3FeF1/J (Stock No. 001022; an F1 strain with the desired functional Tlr4 allele from the C57BL/6J background). Hemizygous mice at The Jackson Laboratory exhibit an approximately 40% survival rate to weaning age.

Related Strains

Strains carrying   Tg(Scgb1a1-Scnn1b)6608Bouc allele

006438	B6.Cg-Tg(Scgb1a1-Scnn1b)6608Bouc/J
006176	B6C3Fe-Tg(Scgb1a1-Scnn1b)6608Bouc/J

View Strains carrying   Tg(Scgb1a1-Scnn1b)6608Bouc     (2 strains)

Strains carrying other alleles of Scgb1a1

006232	B6.Cg-Tg(Scgb1a1-rtTA)1Jaw/J
006242	C.Cg-Tg(Scgb1a1-rtTA)1Jaw/J
006222	FVB.Cg-Tg(Scgb1a1-rtTA)1Jaw/J

View Strains carrying other alleles of Scgb1a1     (3 strains)

Additional Web Information

Genetic Quality Control Annual Report

Research Applications

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

Developmental Biology Research
Internal/Organ Defects (lung)
Perinatal Lethality

Immunology and Inflammation Research
Cystic Fibrosis

Internal/Organ Research
Lung Defects

Mouse/Human Gene Homologs
cystic fibrosis

References

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

---

Price and Supply Information

Strain Name:	B6;C3H-Tg(Scgb1a1-Scnn1b)6608Bouc/J
Stock Number:	005315

Price Details

IMPORTANT NOTE: Prices are based on shipping destination. To view prices, select your shipping destination.

• USA, Canada and Mexico
• International

*NO Shipping Destination selected!

 

Supply Details

Standard Supply	Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes	Cryorecovery - Standard. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that 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. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery. Cryorecovery to establish a Dedicated Supply for greater quantities of mice. One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services: Tel: 1-800-422-6423 or 1-207-288-5845; Email: jaxservices@jax.org. This strain is included in the Induced Mutant Resource Colony collection. Genomic DNA is available for this strain from the Mouse DNA Resource.
Licensing	See General Terms and Conditions below for Licensing and Use Restrictions
Control Information	View Control Information in Strain Details.

General Terms and Conditions

View JAX^® Mice & Services Conditions of Use.

For additional Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.

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.

---

Ordering and Purchasing Information

      Purchasing Information
      JAX^® Mice Orders
      Surgical Services

Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form

Go to JAX^® Mice Query Form