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健康人和一名先天性糖基化障碍患者血清中触珠蛋白的碳水化合物结构

Carbohydrate structures of haptoglobin in sera of healthy people and a patient with congenital disorder of glycosylation.

作者信息

Ferens-Sieczkowska M, Olczak M

机构信息

Department of Chemistry and Immunochemistry, Wrocław Medical University, Poland.

出版信息

Z Naturforsch C J Biosci. 2001 Jan-Feb;56(1-2):122-31. doi: 10.1515/znc-2001-1-219.

DOI:10.1515/znc-2001-1-219
PMID:11302201
Abstract

Haptoglobin is one of acute phase glycoproteins often used as markers in glycopathology studies. In this work the oligosaccharide structures of haptoglobin from 'healthy' subjects have been studied in detail, taking into consideration the possible dependence of glycosylation on the phenotype. About 75% of charged haptoglobin glycans were of biantennary complex structure, and some of them lacked one terminal sialic acid molecule. Triantennary structures made up almost 25% of the charged glycans pool, and highly branched tetrasialylated oligosaccharides did not exceed 1%. The main difference between haptoglobin derived from the sample of pooled 44 sera and from the 2-2 phenotype individual concerned the relative content of trisialylated oligosaccharide with one 2-3 linked sialic acid residue. The oligosaccharide profile of haptoglobin derived from serum of a patient suffering from congenital disorder of glycosylation was compared to 'healthy' controls. It was shown, that four main glycans are identical in patient and 'normal' haptoglobins. Some alterations were found in the relative content of mono-, bi-, and trisialylated glycans as well as in the appearance of some tracely abundant oligosaccharides in haptoglobin of the patient with congenital disorder of glycosylation.

摘要

触珠蛋白是急性期糖蛋白之一,常用于糖病理学研究中的标志物。在这项工作中,我们详细研究了“健康”受试者触珠蛋白的寡糖结构,同时考虑了糖基化可能与表型的相关性。约75%带电荷的触珠蛋白聚糖具有双天线复杂结构,其中一些缺少一个末端唾液酸分子。三天线结构占带电荷聚糖池的近25%,高度分支的四唾液酸化寡糖不超过1%。来自44份混合血清样本的触珠蛋白与2-2表型个体的触珠蛋白之间的主要区别在于带有一个2-3连接唾液酸残基的三唾液酸化寡糖的相对含量。将一名患有糖基化先天性疾病患者血清中的触珠蛋白寡糖谱与“健康”对照进行了比较。结果表明,患者和“正常”触珠蛋白中的四种主要聚糖是相同的。在患有糖基化先天性疾病患者的触珠蛋白中,单唾液酸化、双唾液酸化和三唾液酸化聚糖的相对含量以及一些微量丰富寡糖的出现都发现了一些变化。

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