中国实用儿科杂志

中国实用儿科杂志

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GH-IGF-I轴信号通路及旁分泌信号通路生长障碍发生机制

  

  1. 电子科技大学医学院附属妇女儿童医院  成都市妇女儿童中心医院儿童遗传与内分泌代谢科，四川  成都  611731
  • 出版日期:2021-08-06 发布日期:2021-09-09

GH-IGF-I axis signaling pathway and paracrine signaling pathways in the pathogenesis of growth disorder

  1. Chengdu Women and Children’s Central Hospital，School of Medicine，University of Electronic Science and Technology of China，Chengdu  611731，China
  • Online:2021-08-06 Published:2021-09-09

摘要： 生长激素（growth hormone，GH）被认为是促生长的主要激素。除此之外，还调节碳水化合物、脂类、蛋白质等的代谢。影响胰岛素样生长因子（insulin-like growth factor，IGF）系统的生长激素（GH）-IGF-I轴的人类遗传缺陷，以生长障碍为主要临床特征。GH主要通过激活GH受体（GH receptor，GHR）-JAK2-信号转导子和转录激活子（transducer and activator of transcription-5B，STAT-5B）信号通路来调节IGF-I的产生，从而促进生长发育。多种旁路途径也被GHR-JAK2系统激活，包括ERK1，2（extracellular signal regulated kinases，ERK）和磷酸肌醇-3激酶（phosphoinositide-3 kinase，PI3K）途径。信号通路调节元件，包括SOCS蛋白、磷酸酶和GHR金属蛋白酶也在生长障碍疾病中发挥作用。GHR-JAK2-STAT-5B为GH作用经典通路途径，EKRs通路和IRS-PI3K途径为主要旁路途径。

关键词: 生长激素, 生长激素受体, GH-IGF-I轴, 信号通路, IGF-I受体

Abstract: For many years，growth hormone（GH） has been known to be the primary hormone responsible for body growth.GH also regulates carbohydrate，protein and lipid metabolism. Human genetic defects affecting the GH-insulin-like growth factor（IGF）-I axis of the IGF system present with growth failure as their principal clinical feature. GH promotes postnatal human growth primarily by regulating IGF-I production through activation of the GH receptor （GHR）-JAK2-signal transducer and activator of transcription （STAT）-5B signaling pathway. Many other pathways are activated by the GHR-JAK2 system，including the ERK1，2（extracellular signal regulated kinases，ERK） pathway and the PI3K（phosphoinositide-3 kinase） pathways. Modulating elements，including the SOCS proteins，phosphatases，and GHR metalloproteinase，also play a role in growth failure. GHR-JAK2-STST-5B is a classical pathway，and EKRs and IRS-PI3K are the main bypass pathways.

Key words: growth hormone, growth hormone receptor, GH-IGF-I axis, signaling pathway, IGF-I receptor