Several of the genes known to be essential for sex determination are highly conserved in mammals including human and mouse: WT-128–30, SF-130–32, SOX92,3,33, AMH34,35, whereas mutations for instance in the DAX1 gene lead to adrenal hypoplasia and hypogonadotropic hypogonadism in humans36, with no effect on gonadal development and spermatogenesis in murine Dax1 null mutation37. Here, NR0B1 is linked to chronic primary adrenal insufficiency.