Proposed mechanism of IRX3 oncogenic activation in T-ALL due to the loss of a “promoter tether.” IRX3 resides in a TAD that contains 2 relevant regulatory elements: 1 within intron 8 of FTO and 1 within CRNDE. In cells with wild-type FTO (left panel), the IRX3 promoter is sequestered to a relatively inert region of FTO intron 8, yielding minimal transcriptional activity. This trapping is facilitated by CTCF binding at the FTO intron, which creates an IRX3 “promoter tether.” In T-ALL cells with focal deletions of FTO intron 8 (right panel), the loss of CTCF binding unleashes the IRX3 promoter, allowing it to be hijacked by the distal highly active developmental superenhancer of CRNDE, resulting in oncogenic IRX3 transcription.

Proposed mechanism of IRX3 oncogenic activation in T-ALL due to the loss of a “promoter tether.” IRX3 resides in a TAD that contains 2 relevant regulatory elements: 1 within intron 8 of FTO and 1 within CRNDE. In cells with wild-type FTO (left panel), the IRX3 promoter is sequestered to a relatively inert region of FTO intron 8, yielding minimal transcriptional activity. This trapping is facilitated by CTCF binding at the FTO intron, which creates an IRX3 “promoter tether.” In T-ALL cells with focal deletions of FTO intron 8 (right panel), the loss of CTCF binding unleashes the IRX3 promoter, allowing it to be hijacked by the distal highly active developmental superenhancer of CRNDE, resulting in oncogenic IRX3 transcription.

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