This transgenic strain carries a truncated form of human CFTR (cystic fibrosis transmembrane conductance regulator) under the control of a rat Fabp2 (fatty acid binding protein 2, intestinal) promoter. When crossed with a Cftr knockout mouse the lethal intestinal blockage phenotype associated with cystic fibrosis is rescued and corrected.
Michael Welsh, University of Iowa
Genetic Background | Generation |
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Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
Current therapeutic technologies that apply adeno-associated virus (AAV) gene transfer are limited by viral packaging capacity. Some full-length genes, like Cftr (cystic fibrosis transmembrane conductance regulator) have not been practical candidates for these therapies.
This transgenic strain, carrying a truncated form of human CFTR under the control of a rat Fabp2 (fatty acid binding protein 2, intestinal) promoter, was used to study the potential of shortened genetic segments in the treatment of cystic fibrosis. The N-terminal portion of the regulatory (R) domain was deleted (residues 708-759), but regulated anion channel activity is retained in vitro. When crossed with a Cftr knockout mouse (see Stock No. 002196), the intestinal blockage phenotype associated with cystic fibrosis is rescued and corrected. The gross morphology of both small and large intestines is restored and the excessive mucus observed in the crypts of Cftr knockout mice is reduced.
Nucleotides -1178 to +28 of the rat intestinal fatty acid binding protein (Fabp2) promoter drive expression of a human CFTR cDNA from which sequences encoding N-terminal residues 708-759 (nucleotides 2254-2409) have been deleted (Ref Seq NM_000492). This transgenic strain was created with C57BL/6 x SJL/J F1 embryos, but maintained on a mixed genetic background incorporating C57BL/6, 129, BALB/c, FVB, and SJL genetic contributions by the donating laboratory.
Expressed Gene | CFTR, cystic fibrosis transmembrane conductance regulator, human |
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Site of Expression |
Allele Name | transgene insertion 1, Michael J Welsh |
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Allele Type | Transgenic (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | CFTRdeltaR |
Gene Symbol and Name | Tg(Fabp2-CFTR*)1Wsh, transgene insertion 1, Michael J Welsh |
Gene Synonym(s) | |
Promoter | Fabp2, fatty acid binding protein 2, rat |
Expressed Gene | CFTR, cystic fibrosis transmembrane conductance regulator, human |
Strain of Origin | (C57BL/6 x SJL/J)F1 |
Chromosome | UN |
Molecular Note | Nucleotides -1178 to +28 of the rat intestinal fatty acid binding protein (Fabp2) promoter drive expression of a human CFTR cDNA from which sequences encoding N-terminal residues 708-759 have been deleted. Line 1 was generated. |
Mutations Made By | Michael Welsh, University of Iowa |
Both hemizygotes and homozygotes are viable and fertile, and may be used to maintain a breeding colony.
When using the STOCK Tg(Fabp2-CFTR*)1Wsh/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #017957 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Hemizygous or Non Carrier for Tg(Fabp2-CFTR*)1Wsh |
Frozen Mouse Embryo | STOCK Tg(Fabp2-CFTR*)1Wsh/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | STOCK Tg(Fabp2-CFTR*)1Wsh/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | STOCK Tg(Fabp2-CFTR*)1Wsh/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | STOCK Tg(Fabp2-CFTR*)1Wsh/J Frozen Embryo | $3373.50 |
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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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