In consistence with the emerging concept of vitamin A-dependent diaphragm development, it was shown NR2F2 (COUP-TFII) transcription factors could act as repressors of and in turn be regulated by retinoic acid signalling [65, 66] and by this, a possible mechanism in CDH pathogenesis was revealed. The gene discussed is NR2F2; the disease is congenital diaphragmatic hernia.