Postel-Vinay M C, Finidori J, Goujon L, Martini J F, Delehaye Zervas M C, Kelly P A
Unité INSERM 344, Faculté de Médecine Necker Enfants Malades, Paris.
Ann Endocrinol (Paris). 1991;52(6):469-73.
Growth hormone (GH) exerts multiple actions and GH receptors have been demonstrated in a variety of tissues. Changes in the number of hepatic GH receptors have been demonstrated in several models of growth failure in rats. Cloning of the rabbit and human liver GH receptor has shown that the receptor is a single polypeptide chain of 620 amino acids, made of an extracellular hormone-binding domain, with 7 cysteines and 5 potential glycosylation sites, a unique transmembrane domain and a long cytoplasmic domain. The GH receptor belongs to a new family including prolactin and cytokine receptors. Signal transduction pathways are unknown for these receptors, which do not possess any consensus sequences homologous to tyrosine kinases. The GH-binding protein (GH-BP), identified in plasma of man and other species, corresponds to the extracellular binding domain of the membrane GH receptor. Evaluation of the GH-BP is a direct approach to the GH receptor in man in vivo. Complete absence of GH binding activity has been found in the plasma of patients with Laron dwarfism, in particular those for whom a mutation in the GH receptor gene has been demonstrated.
生长激素（GH）具有多种作用，并且已在多种组织中证实存在GH受体。在大鼠生长衰竭的几种模型中已证实肝脏GH受体数量的变化。兔和人肝脏GH受体的克隆表明，该受体是一条由620个氨基酸组成的单多肽链，由一个细胞外激素结合结构域组成，有7个半胱氨酸和5个潜在的糖基化位点，一个独特的跨膜结构域和一个长的细胞质结构域。GH受体属于一个新的家族，包括催乳素和细胞因子受体。这些受体的信号转导途径尚不清楚，它们不具有与酪氨酸激酶同源的任何共有序列。在人和其他物种的血浆中鉴定出的GH结合蛋白（GH-BP）与膜GH受体的细胞外结合结构域相对应。评估GH-BP是在人体内直接研究GH受体的一种方法。已发现拉伦侏儒症患者的血浆中完全缺乏GH结合活性，尤其是那些已证实GH受体基因发生突变的患者。
