Primary defect of insulin receptors in skin fibroblasts cultured from an infant with leprechaunism and insulin resistance.
Primary defect of insulin receptors in skin fibroblasts cultured from an infant with leprechaunism and insulin resistance.
复制标题
从患有妖精和胰岛素抵抗的婴儿培养的皮肤成纤维细胞中胰岛素受体的主要缺陷。
DOI：
10.1073/pnas.76.11.5877
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发表时间：
1979
影响因子：
11.1
通讯作者：
Alan M. Rosenberg
中科院分区：
文献类型：
--
作者：
Ellen E. Schilling;M. Rechler;Carl GRUNFELDt;Alan M. Rosenberg
与年龄匹配的对照组相比，具有胰岛素抵抗和小矮妖症临床特征的婴儿培养的皮肤成纤维细胞中胰岛素与胰岛素受体的结合明显减少。相比之下，表皮生长因子（一种化学上与胰岛素无关的多肽生长因子）与患者和对照成纤维细胞的结合是难以区分的。胰岛素与患者成纤维细胞结合的选择性缺陷反映在胰岛素刺激2-脱氧葡萄糖摄取的能力受损。这些结果很可能表明胰岛素受体存在原发性遗传缺陷。
Insulin binding to insulin receptors, on skin fibroblasts established in culture from an infant with insulin resistance and clinical features of leprechaunism was markedly decreased in comparison with cultures from an age-matched control. By contrast, the binding of epidermal growth factor, a polypeptide growth factor chemically unrelated to insulin, to patient's and control fibroblasts was indistinguishable. The selective defect in insulin binding to patient's fibroblasts was reflected in an impaired ability of insulin to stimulate 2-deoxyglucose uptake. These results most likely indicate a primary genetic defect of insulin receptors.