HURLER AND HUNTER SYNDROMES - MUTUAL CORRECTION OF DEFECT IN CULTURED FIBROBLASTS
HURLER AND HUNTER SYNDROMES - MUTUAL CORRECTION OF DEFECT IN CULTURED FIBROBLASTS
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DOI：
10.1126/science.162.3853.570
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发表时间：
1968-01-01
期刊：
影响因子：
56.9
通讯作者：
NEUFELD, EF
中科院分区：
文献类型：
--
作者：
FRATANTONI, JC;HALL, CW;NEUFELD, EF
如果将这两种基因型的细胞彼此混合或与正常细胞混合，则可以纠正来自Hurler或Hunter患者的培养皮肤成纤维细胞的生化缺陷（硫酸化粘多糖的错误降解，导致过度的细胞内积累）。这种效应是由释放到培养基中的物质介导的。
The biochemical defect of cultured skin fibroblasts from Hurler or Hunter patients (faulty degradation of sulfated mucopolysaccharide, resulting in excessive intracellular accumulation) may be corrected if cells of these two genotypes are mixed with each other or with normal cells. The effect is mediated by substances released into the medium.