| 粘多糖沉积病的生化研究——Ⅰ、两例Sanfilippo综合征尿氨基多糖的分析 |
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| 引用本文： | 田梦玉,张振强.粘多糖沉积病的生化研究——Ⅰ、两例Sanfilippo综合征尿氨基多糖的分析[J].西安交通大学学报(医学版),1986(3). |
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| 作者姓名： | 田梦玉 张振强 |
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| 作者单位： | 西安医科大学生物化学教研室
(田梦玉)，西安医科大学生物化学教研室(张振强) |
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| 摘 要： | 应用电泳、酶或化学处理及离子交换柱层析分析了我国两例(胞兄妹)Sanfilippo综合征尿氨基多糖的生化特点。两患者的尿氨基多糖主要为硫酸乙酰肝素,分别占71%及58%,而硫酸软骨素则占29%及42%。患者尿硫酸乙酰肝素经纯化后测其C/O比值为2.32,显示其在己糖醛酸组成上与一般硫酸乙酰肝素无明显差异.但DEAE-SephadexA-25柱层析及在0.1M HCl中的电泳分析,均说明患者排泄的硫酸乙酰肝素是不均一的,它至少有两种硫酸基及电荷密度不同的分子类型。
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BIOCHEMICAL STUDIES ON MUCOPOLYSACCHARIDOSIS Ⅰ. CHARACTERIZATION OF GLYCOSAMINOGLYCANS IN URINE FROM TWO PATIENTS WITH SANFILIPPO'S SYNDROME |
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| Tian Mengyu Zhang Zhenqiang.BIOCHEMICAL STUDIES ON MUCOPOLYSACCHARIDOSIS Ⅰ. CHARACTERIZATION OF GLYCOSAMINOGLYCANS IN URINE FROM TWO PATIENTS WITH SANFILIPPO'S SYNDROME[J].Journal of Xi‘an Jiaotong University:Medical Sciences,1986(3). |
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| Authors: | Tian Mengyu Zhang Zhenqiang |
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| Institution: | Department of Biochemistry |
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| Abstract: | Urine glycosaminoglycans of two patients (full brother and sister )with Sanfilippo's syndrome were analysed by electrophoresis, enzymatic or chemical treatment and ion-exchange chromatography. Heparin sulfate is the main glycosaminoglycan accouting for 70% and 58% of urine glycosaminoglycans in both patients, whereas only 29% and 42% of urine glycosaminoglycans are cnondroitin sulfate. Tbe C/O ratio urine heparin sulfate of the patients is 2.32, indicating that the hexuronic acid component parts of the patient's heparin sulfate are much the same as general heparin sulfate. However, the analysis of glycosaminoglycans with DEAE-Sephadex A-25 chromatography and electrophoresis in 0.1 M HCL showed that urine heparin sulfate of the patients arc heterogeneous, and that it covers, at least, two molecular types with different amount of sulfate and charge density. |
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| Keywords: | biochemical analysis urine glycosaminoglycans heparin sulfate sanfilippols syndrome |
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