法布里病成纤维细胞中的糖脂和粘多糖异常。
Glycolipid and mucopolysaccharide abnormality in fibroblasts of fabry's disease.
作者信息
Matalon R, Dorfman A, Dawson G, Sweeley C C
出版信息
Science. 1969 Jun 27;164(3887):1522-3. doi: 10.1126/science.164.3887.1522.
PMID:4239585
Abstract
Cultures of skin fiibroblasts from a patient with Fabry's disease showed an accumulation of the glycolipid, galactosyl-galactosyl-glucosyl ceramide. Such cells also showed metachromasia on staining with toluidine blue and a markedly elevated acid mucopolysaccharide content.
摘要
对一名法布里病患者的皮肤成纤维细胞进行培养，结果显示糖脂半乳糖基 - 半乳糖基 - 葡萄糖基神经酰胺出现蓄积。在用甲苯胺蓝染色时，这些细胞也呈现异染性，并且酸性黏多糖含量显著升高。
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