These mice express a (GAA)230 expansion repeat from the endogenous Fxn locus and do not carry the neo selection cassette. This mutant mouse strain may be useful in studies of Friedreich's ataxia.
Our preclinical efficacy testing services offer scientific expertise and an array of target-based and phenotype-based outcome measures, both in vivo and at endpoint, for flexible study designs and assay development in mouse models of Friedreich's Ataxia. See our full service platform.
IMR Colony, The Jackson Laboratory
Genetic Background | Generation |
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|
Allele Type | Gene Symbol | Gene Name |
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Targeted (Inserted expressed sequence, Humanized sequence) | Fxn | frataxin |
These frataxin (GAA)230Δneo mice harbor the same frataxin (GAA)230 expansion mutation as Stock No. 008470, with the exception that these (GAA)230Δneo mice have had the neo selection cassette removed.
For a description of the frataxin (GAA)230 expansion mutation still harboring the neo cassette, please see Stock No. 008470. The difference in phenotype between the (GAA)230 mice and these (GAA)230Δneo mice, if any, is currently under investigation (January 2011).
To generate these frataxin (GAA)230Δneo animals, mice heterozygous for the frataxin (GAA)230 expansion mutation (Fxntm1Pand) were bred with mice harboring the embryonic cre-expressing mutation Meox2-Cre (Meox2tm1(cre)Sor; Stock No. 003755). Progeny bearing the GAA expansion with the neo selection cassette deleted ((GAA)230Δneo ; Fxntm1.1Pand) were bred together, and the Meox2-Cre mutation was selectively removed.
For a description of the frataxin (GAA)230 expansion mutation (Fxntm1Pand), please see Stock No. 008470.
Allele Name | targeted mutation 1.1, Massimo Pandolfo |
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Allele Type | Targeted (Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | |
Gene Symbol and Name | Fxn, frataxin |
Gene Synonym(s) | |
Promoter | Fxn, frataxin, mouse, laboratory |
Strain of Origin | 129/Sv |
Chromosome | 19 |
Molecular Note | Mice carrying the Fxntm1Pand allele were crossed with mice carrying the Meox2tm1(cre)Sor allele to remove the neomycin cassette, leaving an insertion of a GAA230 repeat expansion. |
Mutations Made By | Massimo Pandolfo, Universite Libre de Bruxelles |
When maintaining a live colony, these mice can be bred as homozygotes.
When using the (GAA)230Δneo mouse strain in a publication, please cite the originating article(s) and include JAX stock #011113 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
---|---|---|
Heterozygous for Fxn<tm1.1Pand> |
Frozen Mouse Embryo | B6.129-Fxn<tm1.1Pand>/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6.129-Fxn<tm1.1Pand>/J Frozen Embryo | $2595.00 |
Frozen Mouse Embryo | B6.129-Fxn<tm1.1Pand>/J Frozen Embryo | $3373.50 |
Frozen Mouse Embryo | B6.129-Fxn<tm1.1Pand>/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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