Brue T, Vallette S, Pellegrini-Bouiller I, Enjalbert A
Laboratoire ICNE-UMR 6544, Institut Jean-Roche, Faculté de Médecine Nord, Marseille.
Ann Endocrinol (Paris). 1997;58(6):436-50.
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.
在垂体中已证实的一些转录因子可能在该腺体的发育中起作用，即垂体特异性转录因子1（Pit-1）、先知因子1（Prop-1）、P-Lim、配对盒基因1（Ptx1）、视网膜前同源盒蛋白（Rpx）、剂量敏感的性反转-先天性肾上腺皮质增生-先天性肾上腺发育不全综合征基因1（Dax-1）、类固醇生成因子1（SF-1）。其中一些因子与垂体功能减退的动物模型有关，而迄今为止，仅显示垂体特异性转录因子1基因改变是人类垂体功能减退的原因。简要介绍这些因子，并综述目前关于基因决定的单激素或多激素垂体功能减退的数据。特别是，详细介绍了由于垂体特异性转录因子1基因改变导致的生长激素细胞、催乳激素细胞和促甲状腺激素细胞联合缺乏的数据。分析该综合征以及其他多种垂体激素缺乏病理模型中的表型-基因型关系，将为有助于垂体腺发育以及不同垂体细胞系分化和调节的复杂事件序列提供有用信息。
