[Endocrinology 1994-1995]

In a brief review of advances made in endocrinology during the past two years surprisingly the receptors for TSH and thyroid hormones occupy the foremost position. Mutations of the TSH receptor may cause congenital hyperthyroidism or resistance to TSH (mutation of the gene for TSH-receptor). An interaction of thyroid hormone receptor with a conserved transcriptional mediator was discovered. The reports on the treatment of neonatal respiratory distress syndrome with TRH are still controversial. CRH deficiency reveals neonatal but not adult glucocorticoid need--at least in mice. The concept of receptors was challenged by the discovery of "inverse agonists", i.e. ligand binding by (already) activated receptors. To make endocrinology even more complicated it was shown, that the congenital lipoid adrenal hyperplasia may be caused not by a deficiency in an adrenal cortex enzyme, but by a mutation of the mitochondrial transport protein (StAR), steroidogenic acute protein). The gene for X-linked congenital adrenal hypoplasia (DAX-1) was isolated, its mutation can cause a hypogonadotropic hypogonadism, too. Animals lacking functional estrogen receptor develop osteoporosis--this is true even in humans. Nitric oxide (NO) has more jobs in the body than previously thought-e.g. for maintenance of diurnal rhythms. The interest in endogenous cannabinoids is still flourishing, especially in the brain. Clinical implications of basic research are best documented by the discoveries of G-protein roles in clinical syndromes (e.g. pseudohypoparathyroidism, McCune-Albright syndrome, acromegaly and "testitoxicosis"). It should be emphasized that Nobel prize for medicine and physiology for the year 1994 was awarded to A. G. Gilman and M. Rodbell for the discovery of G-proteins.