CONGENITAL MULTIPLE ANTERIOR-PITUITARY HO RMONE DEFICIENCIES - AN APPROACH TO PITUITARY ONTOGENY

A number of transcription factors evidenced in the pituitary may play a part in the development of this gland, namely Pit-1, Prop-1, P-Lim, Ptx1, Rpx, Dax-1, SF-1. Several of these factors are involved in anima l models of hypopituitarism, while up to now only Pit-1 gene alteratio ns have been shown to be responsible for hypopituitarism in man. These factors are briefly presented, and current data on genetically determ ined uni- or multi-hormonal pituitary deficiencies are reviewed. In pa rticular, data on combined somatotroph, lactotroph and thyrotroph defi ciencies due to Pit-1 gene alterations are detailed. Analysis of pheno type-genotype relationships in this syndrome and in other pathological models of multiple pituitary hormone deficiencies will provide useful information on the complex sequence of events that contribute to the development of pituitary gland and to differentiation and regulation o f the different pituitary cell lines.