FIG. 1.

(A) The PAX3-FKHR chimeric transcription factor generated by the t(2;13) translocation in alveolar rhabdomyosarcoma (ARMS) retains the PAX3 paired box (PB) and homeodomain (HD) DNA binding motifs, has a disrupted forkhead (FD) DNA binding domain, and acquires a COOH-terminal FKHR-derived activation domain. (B) An engineered transcriptional repressor-corepressor recruitment strategy for inhibiting oncogenic activator function. The PAX3-KRAB repressor was designed to directly compete with PAX3-FKHR for binding and silence PAX3 target genes.