Advances in glycosaminoglycan detection.

Autor: Khan SA; Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA., Mason RW; Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA; Department of Biological Sciences, University of Delaware, Newark, DE, USA., Kobayashi H; Department of Pediatrics, Shimane University, Shimane, Japan., Yamaguchi S; Department of Pediatrics, Shimane University, Shimane, Japan., Tomatsu S; Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA; Department of Pediatrics, Shimane University, Shimane, Japan; Department of Pediatrics, Graduate School of Medicine, Gifu University, Gifu, Japan; Department of Pediatrics, Thomas Jefferson University, Philadelphia, PA, USA. Electronic address: stomatsu@nemours.org.
Jazyk: angličtina
Zdroj: Molecular genetics and metabolism [Mol Genet Metab] 2020 Jun; Vol. 130 (2), pp. 101-109. Date of Electronic Publication: 2020 Mar 27.
DOI: 10.1016/j.ymgme.2020.03.004
Abstrakt: 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.
(Copyright © 2020 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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