[Congenital multiple anterior pituitary hormone deficiencies. An approach of pituitary ontogenesis].
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 animal models of hypopituitarism, while up to now only Pit-1 gene alterations have been shown to be responsible for hypopituitarism in man. These factors are briefly presented, and current data on genetically determined uni- or multi-hormonal pituitary deficiencies are reviewed. In particular, data on combined somatotroph, lactotroph and thyrotroph deficiencies due to Pit-1 gene alterations are detailed. Analysis of phenotype-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 the pituitary gland and to differentiation and regulation of the different pituitary cell lines.
Laboratoire ICNE-UMR 6544, Institut Jean-Roche, Faculté de Médecine Nord, Marseille.
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Brue
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Vallette
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Pellegrini-Bouiller
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Enjalbert
D000818:Animals; D004268:DNA-Binding Proteins / Q000235:genetics; D004195:Disease Models, Animal; D018507:Gene Expression Regulation, Developmental; D018398:Homeodomain Proteins / Q000235:genetics; D007018:Hypopituitarism / Q000151:congenital* / Q000235:genetics / Q000503:physiopathology; D051379:Mice; D010641:Phenotype; D010903:Pituitary Gland, Anterior / Q000196:embryology* / Q000378:metabolism / Q000473:pathology; D010908:Pituitary Hormones, Anterior / Q000172:deficiency*; D050817:Transcription Factor Pit-1; D014157:Transcription Factors / Q000235:genetics*
D004268:DNA-Binding Proteins; D018398:Homeodomain Proteins; D010908:Pituitary Hormones, Anterior; D050817:Transcription Factor Pit-1; D014157:Transcription Factors; C494185:Pou1f1 protein, mouse