University Lille, CNRS, UMR 8576 - UGSF - Unité de Glycobiologie Structurale et Fonctionnelle, F-59000, Lille, France.
J Inherit Metab Dis. 2018 May;41(3):515-523. doi: 10.1007/s10545-017-0118-3. Epub 2018 Jan 2.
The development of metabolic oligosaccharide engineering (MOE) over the past two decades enabled the bioimaging studies of glycosylation processes in physio-pathological contexts. Herein, we successfully applied the chemical reporter strategy to image the fate of sialylated glycoconjugates in healthy and sialin-deficient patient fibroblasts. This chemical glycomics enrichment is a powerful tool for tracking sialylated glycoconjugates and probing lysosomal recycling capacities. Thus, such strategies appear fundamental for the characterization of lysosomal storage diseases.
过去二十年中代谢寡糖工程(MOE)的发展使人们能够在生理病理环境下对糖基化过程进行生物成像研究。在此,我们成功地应用化学报告策略对健康和唾液酸酶缺乏症患者成纤维细胞中唾液酸化糖缀合物的命运进行了成像。这种化学糖组学富集是跟踪唾液酸化糖缀合物和探测溶酶体回收能力的有力工具。因此,这种策略似乎对于溶酶体贮积病的特征描述至关重要。