de Jong J G, Wevers R A, Liebrand-van Sambeek R
Laboratory of Paediatrics and Neurology, University Hospital Nijmegen, The Netherlands.
Clin Chem. 1992 Jun;38(6):803-7.
Earlier we described a simple and reliable screening procedure in urine for mucopolysaccharidoses based on the color reaction of glycosaminoglycans (GAGs) with dimethylmethylene blue. At physiological concentrations of urinary protein, we observed an obvious interference by protein in the assay. By modifying the assay, we abolished the protein interference. The modified procedure is not disturbed by human serum albumin, IgG (both tested with as much as 5 g/L of protein), or urinary proteins. The modified procedure appeared as reliable as the original. No false-negative results were found in a series of 26 urine samples from patients with mucopolysaccharidoses (sensitivity 100%). In a series of 405 urine samples offered for metabolic screening, 24 samples with increased GAG content and normal GAG composition were seen (specificity 94%). The method may also be applicable for determining GAG in other body fluids or solutions containing protein.
此前，我们描述了一种基于糖胺聚糖（GAGs）与二甲基亚甲蓝的颜色反应，用于尿液中黏多糖贮积症的简单可靠的筛查程序。在尿蛋白的生理浓度下，我们观察到蛋白质在该检测中产生明显干扰。通过对检测方法进行改进，我们消除了蛋白质干扰。改进后的程序不受人血清白蛋白、IgG（两者均以高达5 g/L的蛋白质进行测试）或尿蛋白的干扰。改进后的程序与原程序一样可靠。在一系列来自黏多糖贮积症患者的26份尿液样本中未发现假阴性结果（敏感性100%）。在提供用于代谢筛查的405份尿液样本中，发现24份样本的GAG含量增加且GAG组成正常（特异性94%）。该方法也可适用于测定其他体液或含蛋白质溶液中的GAG。
