Gt(ROSA)26Sor^{tm1.1(DUX4*)Plj}
Targeted Allele Detail

Summary

• Symbol: Gt(ROSA)26Sor^{tm1.1(DUX4*)Plj}
• Name: gene trap ROSA 26, Philippe Soriano; targeted mutation 1.1, Peter Jones
• MGI ID: MGI:5707276
• Synonyms: FLExDUX4
• Gene: Gt(ROSA)26Sor Location: Chr6:113044389-113054205 bp, - strand Genetic Position: Chr6, 52.73 cM
• Alliance: Gt(ROSA)26Sor^{tm1.1(DUX4*)Plj} page

Mutation origin

• Germline Transmission: Earliest citation of germline transmission: J:256652
• Parent Cell Line: Not Specified (ES Cell)
• Strain of Origin: C57BL/6

Mutation description

• Allele Type: Targeted (Conditional ready, Humanized sequence, Inserted expressed sequence)
• Mutations: Insertion, Nucleotide substitutions
• Mutation details: The targeting vector was designed with (from 5 to 3) a loxP site, an FRT-flanked neomycin resistance gene, a lox511 site, an inverted human double homeobox 4 (DUX4) gene with a polyadenylation sequence, and another loxP site and lox511 site, both in opposite orientation to the 5 sites. The DUX4 gene contains 4 silent point mutations in exon 1 that prevent alternative splicing of a truncated isoform, DUX4-s, while maintaining the DUX4-fl ORF. This entire construct was inserted between exons 1 and 2 of the Gt(ROSA)26Sor locus. Flp-mediated recombination removed the FRT-flanked neo cassette. (J:256652)

Disease models

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 1 strain available Cell Lines: 0 lines available
• Carrying any Gt(ROSA)26Sor Mutation: 993 strains or lines available

References

• Original: J:256652 Jones T, et al., A cre-inducible DUX4 transgenic mouse model for investigating facioscapulohumeral muscular dystrophy. PLoS One. 2018;13(2):e0192657
• All: 7 reference(s)