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.
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IMR Colony, The Jackson Laboratory
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|
|Allele Type||Targeted (Inserted expressed sequence, Humanized sequence)|
|Gene Symbol and Name||Fxn, frataxin|
|Promoter||Fxn, frataxin, mouse, laboratory|
|Strain of Origin||129/Sv|
|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.