Chou L, Nadler H
Pediatr Res. 1976 Mar;10(3):176-8. doi: 10.1203/00006450-197603000-00006.
A serum factor was demonstrated in normal individuals which (1) enhances the incorporation of L-[3H]fucose into cultured human skin fibroblasts, (2) is nondialyzable, (3) is heat labile at 50 degrees, (4) is present in the noneuglobulin fraction, and (5) appears to be deficient in serum from patients with cystic fibrosis. The specific activity of L-[3H]fucose incorporated into skin fibroblasts from normal individuals in the presence of serum from 12 control subjects was 3,337 +/- 168 cpm/mg protein in contrast to 2,294 +/- 172, the activity obtained either in the presence of serum from 10 age-matched patients with cystic fibrosis or in the absence of serum. These differences were significant at P less than 0.001. In comparison, no significant difference was detected in the amount of L-[3H]fucose incorporated into skin fibroblasts derived from normal individuals and patients with cystic fibrosis. The plasma membrane of cultured skin fibroblasts derived from patients with cystic fibrosis appears to be grossly unaltered in its protein and L-fucose labeling pattern.
在正常个体中发现了一种血清因子，它具有以下特性：（1）增强L-[3H]岩藻糖掺入培养的人皮肤成纤维细胞的能力；（2）不可透析；（3）在50摄氏度时对热不稳定；（4）存在于非球蛋白组分中；（5）在囊性纤维化患者的血清中似乎缺乏。在来自12名对照受试者的血清存在下，正常个体皮肤成纤维细胞中掺入的L-[3H]岩藻糖的比活性为3337±168 cpm/mg蛋白质，相比之下，在来自10名年龄匹配的囊性纤维化患者的血清存在下或无血清时获得的活性为2294±172。这些差异在P小于0.001时具有统计学意义。相比之下，在正常个体和囊性纤维化患者来源的皮肤成纤维细胞中，掺入的L-[3H]岩藻糖的量没有检测到显著差异。来自囊性纤维化患者的培养皮肤成纤维细胞的质膜在其蛋白质和L-岩藻糖标记模式上似乎没有明显改变。
