Gt(ROSA)26Sor^{tm1(HD*103Q)Xwy}
Targeted Allele Detail

Summary

• Symbol: Gt(ROSA)26Sor^{tm1(HD*103Q)Xwy}
• Name: gene trap ROSA 26, Philippe Soriano; targeted mutation 1, X William Yang
• MGI ID: MGI:3583991
• Synonyms: mhtt-exon1 (103Q), RosaHD
• Gene: Gt(ROSA)26Sor Location: Chr6:113044389-113054205 bp, - strand Genetic Position: Chr6, 52.73 cM
• Alliance: Gt(ROSA)26Sor^{tm1(HD*103Q)Xwy} page

Mutation origin

• Germline Transmission: Earliest citation of germline transmission: J:99759
• Parent Cell Line: R1 (ES Cell)
• Strain of Origin: (129X1/SvJ x 129S1/Sv)F1-Kitl⁺

Mutation description

• Allele Type: Targeted (Conditional ready, Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Gt(ROSA)26Sor^{tm1(HD*103Q)Xwy} expresses 1 gene
  Knock-in expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  HTT (3064)

  Htt	(MGI:96067)

• Mutation details: A targeting vector was designed with a loxP-flanked transcriptional STOP sequence immediately upstream of a neuropathogenic polyQ-mutant form of the human Huntingtin protein exon 1 (mhtt-exon1) sequence (containing 103 mixed CAA-CAG repeats, each encoding glutamine), all followed by a polyadenylation sequence. When these mice are bred with animals expressing cre under the control of a promoter of interest, The STOP signal is deleted in tissue of interest allowing conditional expression of mhtt1-exon1. (J:99759, J:123199)

Disease models

Expression

In Structures Affected by this Mutation:

2 anatomical structure(s)

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:99759 Gu X, et al., Pathological cell-cell interactions elicited by a neuropathogenic form of mutant Huntingtin contribute to cortical pathogenesis in HD mice. Neuron. 2005 May 5;46(3):433-44
• All: 5 reference(s)