The activation of the FXRα receptor in Leydig cells induces transcription of the gene encoding the orphan nuclear receptors SHP (Small Heterodimer Partner, Nr0b2) and DAX-1 (dosage-sensitive sex reversal, adrenal hypoplasia critical region, on chromosome X, gene 1, Nr0b1), two orphan nuclear receptors, which are negative regulators of steroidogenic Leydig cells activity. The gene discussed is ESRRB; the disease is chronic primary adrenal insufficiency.