BACKGROUND Glycosaminoglycans (GAGs) are negatively charged long linear (highly sulfated) polysaccharides consisting of repeating disaccharide units that are expressed on the surfaces of all nucleated cells. The expression of GAGs is required for embryogenesis, regulation of cell growth and proliferation, maintenance of tissue hydration, and interactions of the cells via receptors. Mucopolysaccharidoses (MPS) are caused by deficiency of specific lysosomal enzymes that result in the accumulation of GAGs in multiple tissues leading to organ dysfunction. Therefore, GAGs are important biomarkers for MPS. Without any treatment, patients with severe forms of MPS die within the first two decades of life. SCOPE OF REVIEW Accurate measurement of GAGs is important to understand the diagnosis and pathogenesis of MPS and to monitor therapeutic efficacy before, during, and after treatment of the disease. This review covers various qualitative and quantitative methods for measurement of GAGs, including dye specific, thin layer chromatography (TLC), capillary electrophoresis, high-performance liquid chromatography (HPLC), liquid chromatography-tandem mass spectrometry (LC-MS/MS), gas chromatography, ELISA, and automated high-throughput mass spectrometry. Major conclusion: There are several methods for GAG detection however, specific GAG detection in the various biological systems requires rapid, sensitive, specific, and cost-effective methods such as LC-MS/MS. GENERAL SIGNIFICANCE This review will describe different methods for GAG detection and analysis, including their advantages and limitation.

中文翻译：

---

糖胺聚糖检测的进展。

背景技术糖胺聚糖（GAG）是带负电荷的长直链（高度硫酸化）多糖，由在所有有核细胞表面表达的重复二糖单元组成。GAG的表达对于胚胎发生，调节细胞生长和增殖，维持组织水合作用以及细胞通过受体的相互作用是必需的。粘多糖酶（MPS）是由特定溶酶体酶的缺乏引起的，这些酶导致GAG在多个组织中积累，从而导致器官功能障碍。因此，GAG是MPS的重要生物标志物。未经任何治疗，患有严重形式MPS的患者会在生命的前二十年内死亡。综述的范围准确测量GAG对了解MPS的诊断和发病机理以及监测疾病治疗之前，之中和之后的治疗效果非常重要。这篇综述涵盖了各种GAG的定性和定量方法，包括染料特异性，薄层色谱（TLC），毛细管电泳，高效液相色谱（HPLC），液相色谱-串联质谱（LC-MS / MS），气相色谱，ELISA和自动高通量质谱分析。主要结论：有几种检测GAG的方法，但是，在各种生物系统中进行特异性GAG的检测需要快速，灵敏，特异且具有成本效益的方法，例如LC-MS / MS。