Absence of serum growth hormone binding protein in patients with growth hormone receptor deficiency (Laron dwarfism).
Absence of serum growth hormone binding protein in patients with growth hormone receptor deficiency (Laron dwarfism).
复制标题
生长激素受体缺乏症（拉伦侏儒症）患者血清生长激素结合蛋白缺失。
DOI：
10.1073/pnas.84.13.4636
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发表时间：
1987
影响因子：
11.1
通讯作者：
Trivedi,B
中科院分区：
文献类型：
--
作者：
Daughaday,WH;Trivedi,B
最近已经认识到，人类血清中含有一种特异性结合人类生长激素（hGH）的蛋白质。该蛋白对生长激素具有与膜结合生长激素受体相同的限制性特异性。为了确定GH结合蛋白是GH受体的衍生物，还是与GH受体相关，我们检查了3例laron型侏儒症患者的血清。laron型侏儒症患者的GH难愈性归因于GH受体的缺陷。125i标记的生长激素与血清孵育后，通过Ultrogel AcA 44微型柱对血清进行凝胶过滤，测定其结合情况。当125I-hGH与过量生长激素存在的血清孵育时，确定非特异性结合。结果表示为特异性结合125I-hGH的百分比，以及内源性GH校正后相对于参考血清的特异性结合。8例正常成人（26 ~ 46岁）血清特异性结合的平均+/- SEM为21.6 +/- 0.45%，相对特异性结合为101.1 +/- 8.6%。11例正常儿童血清特异性结合较低，为12.5 +/- 1.95%，相对特异性结合为56.6 +/- 9.1%。3名laron型侏儒症患儿的血清缺乏任何明显的GH结合，而另外10名其他类型的非垂体性矮小患儿的血清具有正常的相对特异性结合。我们认为血清GH结合蛋白是GH受体的可溶性衍生物。测定血清GH结合蛋白可以识别GH受体的其他异常。
It has recently been recognized that human serum contains a protein that specifically binds human growth hormone (hGH). This protein has the same restricted specificity for hGH as the membrane-bound GH receptor. To determine whether the GH-binding protein is a derivative of, or otherwise related to, the GH receptor, we have examined the serum of three patients with Laron-type dwarfism, a condition in which GH refractoriness has been attributed to a defect in the GH receptor. The binding of 125I-labeled hGH incubated with serum has been measured after gel filtration of the serum through an Ultrogel AcA 44 minicolumn. Nonspecific binding was determined when 125I-hGH was incubated with serum in the presence of an excess of GH. Results are expressed as percent of specifically bound 125I-hGH and as specific binding relative to that of a reference serum after correction is made for endogenous GH. The mean +/- SEM of specific binding of sera from eight normal adults (26-46 years of age) was 21.6 +/- 0.45%, and the relative specific binding was 101.1 +/- 8.6%. Sera from 11 normal children had lower specific binding of 12.5 +/- 1.95% and relative specific binding of 56.6 +/- 9.1%. Sera from three children with Laron-type dwarfism lacked any demonstrable GH binding, whereas sera from 10 other children with other types of nonpituitary short stature had normal relative specific binding. We suggest that the serum GH-binding protein is a soluble derivative of the GH receptor. Measurement of the serum GH-binding protein may permit recognition of other abnormalities of the GH receptor.