PROGRAM PROJECT IN THE PATHOPHYSIOLOGY OF HUMAN GROWTH

该方案项目长期目标是 儿童正常成长但未能实现的机制 身材正常。遵循传统临床分类 特征、生长激素和IGF-I分泌标准：(1)潜力 将寻求hGH和IGF I基因的突变，并在 围绕这些基因进行了研究，并对这些基因的调控进行了研究。 (2)循环中hGH和IGF多肽的结构异常 表征及其受体亲和力和生物活性 量化的。(3)描述hGH受体的结构缺陷 以及IGF的寡糖单元结构、生物合成和周转 确定了受体。(4)可能的受体后异常事件 定义，以及(5)体内意义和临床后果 这些干扰将用多方面的稳定同位素进行研究。 蛋白质合成代谢和分解代谢对外源激素反应的动力学探针 生长激素注射。

The long-term objective of this program project is to characterize mechanisms by which children both grow appropriately and fail to achieve normal stature. Following classification by conventional clinical characteristics, growth hormone and IGF I secretory criteia: (1) potential mutations in hGH and IGF I genes will be sought, polymorphism within and around these genes examined, and the regulation of these genes studied. (2) Structural abnormalities in circulating hGH and IGF peptides will be characterized and their receptor affinities and biologic activities quantified. (3) Structural defects of the hGH receptor will be delineated and the oligosaccharide unit structure, biosynthesis, and turnover of IGF receptors determined. (4) Possible aberrant post-receptor events will be defined, and (5) The in vivo significance and clinical consequences of these disturbances will be investigated with multi-faceted stable-isotope kinetic probes of protein anabolic and catabolic responses to exogenous growth hormone administration.