For example, NR0B1 (encoded by the Nr0b1 gene), also known as the dosage-sensitive sex reversal, adrenal hypoplasia congenital critical region on the X-chromosome, gene 1 (Dax1), is an orphan nuclear receptor regulating the expression of steroidogenic enzymes in mice23, 24. Here, ESRRB is linked to chronic primary adrenal insufficiency.