Steroid hormones are essential for normal sexual development, accommodation
to stress, and regulation of fluid and electrolyte balance. Biosynthesis o
f these different classes of steroids and its appropriate regulation requir
es the precisely controlled expression of six different cytochrome P-450 en
zymes and two hydroxysteroid dehydrogenases in different tissues. The molec
ular mechanism of the tissue-specific and pituitary hormone-regulated expre
ssion of the genes encoding P-450 enzymes in the steroidogenic tissues is t
he central problem of long-term regulation of steroidogenesis. Orphan membe
rs of the nuclear receptor superfamily play an important role in mediating
transcriptional regulation of several steroid hydroxylase genes. Two of the
se transcription factors, steroidogenic factor-1 (SF-1) and DAX-1, will be
reviewed here in detail.