Purkiss P, Gibbs D A, Watts R W
Clin Chim Acta. 1983 Jun 30;131(1-2):109-21. doi: 10.1016/0009-8981(83)90357-1.
This communication reports studies on the composition of the urinary glycosaminoglycans and oligosaccharides in mucopolysaccharidosis patients who were being treated by fibroblast transplantation. The urinary glycosaminoglycans were precipitated with 9-aminoacridine, the oligosaccharides remaining in solution. Both fractions were further subfractionated by gel filtration. The glycosaminoglycan subfractions were examined for their content of iduronic acid, glucuronic acid, galactosamine and glucosamine. We found no changes in these parameters in a patient who had been treated by repeated fibroblast transplantations over the course of 4 1/2 years. The amino sugar composition of the oligosaccharide fraction was examined and shown to be unchanged. We also found no changes in the degree of sulphation of the urinary glycosaminoglycans specifically related to the transplant in four patients with Hurler disease and two with Hunter disease. We conclude that fibroblast transplantation does not produce detectable changes in the carbohydrate content or degree of sulphation of the urinary glycosaminoglycans and oligosaccharides.
本通讯报道了对接受成纤维细胞移植治疗的黏多糖贮积症患者尿中糖胺聚糖和寡糖成分的研究。尿糖胺聚糖用9-氨基吖啶沉淀，寡糖则留在溶液中。两个组分都通过凝胶过滤进一步细分。对糖胺聚糖亚组分检测其艾杜糖醛酸、葡萄糖醛酸、半乳糖胺和葡萄糖胺的含量。我们发现，在4年半的时间里接受多次成纤维细胞移植治疗的一名患者中，这些参数没有变化。对寡糖组分的氨基糖成分进行检测，结果显示未发生改变。我们还发现，在4例Hurler病患者和2例Hunter病患者中，与移植特异性相关的尿糖胺聚糖硫酸化程度没有变化。我们得出结论，成纤维细胞移植不会使尿糖胺聚糖和寡糖的碳水化合物含量或硫酸化程度产生可检测到的变化。
