In alveolar rhabdomyosarcoma (ARMS), the orphan nuclear receptor 4A1 (NR4A1) is responsible for G9a overexpression by complexing with the Sp1 transcription factor (Sp1) and occupying the -511 GC-rich region of the G9a promoter [61]. This evidence concerns the gene EHMT2 and rhabdomyosarcoma.