Glycosaminoglycans of skin and urine in pseudoxanthoma elasticum: evidence for chondroitin 6-sulfate alteration.
Glycosaminoglycans of skin and urine in pseudoxanthoma elasticum: evidence for chondroitin 6-sulfate alteration.
复制标题
弹性假黄瘤中皮肤和尿液的糖胺聚糖：6-硫酸软骨素改变的证据。
DOI：
10.1016/0009-8981(86)90242-1
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发表时间：
1986
期刊：
影响因子：
--
通讯作者：
Fleischmajer,R
中科院分区：
文献类型：
--
作者：
Longas,MO;Wisch,P;Lebwohl,MG;Fleischmajer,R
从1例严重弹性假黄瘤患者的皮损中分离到异常升高的透明质酸和硫酸皮肤素。这些糖胺多聚糖(估计为糖醛酸)分别比正常对照组高出33.6和4.8个数量级。同一患者的尿6-硫酸软骨素经醋酸纤维素电泳后，其移动速度快于从另外4名患者或正常对照组的尿液中分离出的相应的硫酸软骨素。透明质酸和6-硫酸软骨素分别比正常尿液中的相应物质高出2到10倍和4到18倍。这些增加与所研究的5名患者中的3名患者的弹性假黄瘤的严重程度相关。数据显示：第一个确凿的证据表明，1名患有严重弹性假黄瘤的患者皮损中硫酸皮肤素增加，该患者的透明质酸显著增加，同一患者的尿6-硫酸软骨素改变，以及假性弹性黄瘤患者尿中多种糖胺聚糖的变化。
Abnormally elevated hyaluronic acid and dermatan sulfate were isolated from lesional skin of a patient with severe pseudoxanthoma elasticum. These glycosaminoglycans (estimated as uronic acid) surpassed the normal controls by 33.6 and 4.8 magnitudes, respectively. Urine chondroitin 6-sulfate of the same patient moved faster by electrophoresis on cellulose acetate than its counterpart isolated from urine of another four patients or from the normal controls. Hyaluronic acid and chondroitin 6-sulfate exceeded their counterparts in normal urine by 2- to 10- and 4-to 18-folds, respectively. These increments correlated with the pseudoxanthoma elasticum severity in three of the five patients studied.The data showed: the first conclusive evidence that dermatan sulfate increased in lesional skin of a patient, with severe pseudoxanthoma elasticum, who also had considerably augmented HA, alteration of the same patient's urine chondroitin 6-sulfate, and diversified urine glycosaminoglycans in pseudoxanthoma elasticum.