人源N-乙酰半乳糖胺-6-硫酸酯硫酸酯酶。黏多糖贮积症Ⅳ型中该酶的缺失。
N-acetylgalactosamine-6-sulfate sulfatase in man. Absence of the enzyme in Morquio disease.
作者信息
Singh J, Di Ferrante N, Niebes P, Tavella D
出版信息
J Clin Invest. 1976 Apr;57(4):1036-40. doi: 10.1172/JCI108345.
Abstract
Human N-acetylgalactosamine-6-sulfate sulfatase (6-sulfatase) activity is measured by using as a substrate a sulfated tetrasaccharide obtained by digesting purified chondroitin-6-sulfate (C-6-S) with testicular hyaluronidase. The amount of inorganic sulfate released is measured turbidimetrically. The enzyme from human kidney has a pH optimum of 4.8; its activity is augmented by low levels of NaCl and inhibited by phosphate and high levels of NaCl. Free glucuronate, acetylgalactosamine, inorganic sulfate, polymeric C-6-S, or tetrasaccharide obtained from chondroitin-4-sulfate do not affect the enzyme activity. The method may be used for the diagnosis of Morquio disease since extracts of Morquio fibroblasts are devoid of 6-sulfatase activity.
摘要
人N-乙酰半乳糖胺-6-硫酸酯硫酸酯酶（6-硫酸酯酶）活性的测定方法是，使用一种硫酸化四糖作为底物，该硫酸化四糖是通过用睾丸透明质酸酶消化纯化的硫酸软骨素-6-硫酸酯（C-6-S）获得的。通过比浊法测量释放的无机硫酸盐的量。人肾中的该酶最适pH为4.8；其活性在低水平NaCl存在时增强，而受磷酸盐和高水平NaCl抑制。游离葡萄糖醛酸、乙酰半乳糖胺、无机硫酸盐、聚合C-6-S或从硫酸软骨素-4-硫酸酯获得的四糖不影响该酶的活性。该方法可用于诊断黏多糖贮积症IV型，因为黏多糖贮积症IV型成纤维细胞的提取物缺乏6-硫酸酯酶活性。
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