Simulation of genetic mucopolysaccharidoses in normal human fibroblasts by alteration of pH of the medium.
Simulation of genetic mucopolysaccharidoses in normal human fibroblasts by alteration of pH of the medium.
复制标题
通过改变培养基的 pH 值来模拟正常人成纤维细胞中的遗传性粘多糖增多症。
DOI：
10.1073/pnas.69.9.2361
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发表时间：
1972
影响因子：
11.1
通讯作者：
E. Neufeld
中科院分区：
文献类型：
--
作者：
S. Lie;V. McKusick;E. Neufeld
随着生长培养基的pH从6.8升高至8.0，培养物中正常人成纤维细胞对硫酸化粘多糖的催化作用逐渐受到抑制。最终的细胞密度随着pH值的变化而增加。通过降低pH值，降解粘多糖的能力迅速恢复，并且这种再活化不需要蛋白质合成。在来自具有粘多糖降解的遗传损伤（例如Hurler或Hunter综合征）的患者的细胞中未观察到这种pH依赖性。这些结果可能不仅与细胞培养中粘多糖代谢的研究有关，而且与异染性作为遗传标记的使用有关，并且与观察到正常成纤维细胞随着生长培养基的pH升高而从生长的接触抑制中释放有关。
Catabolism of sulfated mucopolysaccharide by normal human fibroblasts in culture is progressively inhibited as the pH of the growth medium is raised from 6.8 to 8.0. The final cell density increases with the change in pH. The capacity to degrade mucopolysaccharide is rapidly restored by lowering the pH, and this reactivation does not require protein synthesis. Such pH dependence is not observed in cells from patients with genetic impairment of mucopolysaccharide degradation, such as the Hurler or Hunter syndromes. These results may have relevance not only to studies of mucopolysaccharide metabolism in cell culture, but also to the use of metachromasia as a genetic marker and to the observation that normal fibroblasts are released from contact inhibition of growth as the pH of the growth medium is raised.