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    N‑Glycosylation of Serum IgG and Total Glycoproteins in MAN1B1 Deficiency

    Saldova, Radka and Stockmann, Henning and O'Flaherty, Roisin and Lefeber, Dirk J and Jaeken, Jaak and Rudd, Pauline M. (2015) N‑Glycosylation of Serum IgG and Total Glycoproteins in MAN1B1 Deficiency. Journal of Proteome Research, 14 (10). pp. 4402-4412. ISSN 1535-3893

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    MAN1B1-CDG has recently been characterized as a type II congenital disorder of glycosylation (CDG), disrupting not only protein N-glycosylation but also general Golgi morphology. Using our high-throughput, quantitative ultra-performance liquid chromatography assay, we achieved a detailed characterization of the glycosylation changes in both total serum glycoproteins and isolated serum IgG from ten previously reported MAN1B1-CDG patients. We have identified and quantified novel hybrid high-mannosylated MAN1B1-CDG-specific IgG glycans and found an increase of sialyl Lewis x (sLex) glycans on serum proteins of all patients. This increase in sLex has not been previously reported in any CDG. These findings may provide insight into the pathophysiology of this CDG.

    Item Type: Article
    Keywords: MAN1B1; N-glycans; glycomics; ultra performance liquid chromatography; biomarkers; human serum; IgG; CDG; sialyl Lewis x;
    Academic Unit: Faculty of Science and Engineering > Chemistry
    Item ID: 15035
    Identification Number:
    Depositing User: Roisin O'Flaherty
    Date Deposited: 16 Nov 2021 16:10
    Journal or Publication Title: Journal of Proteome Research
    Publisher: American Chemical Society
    Refereed: Yes
    Use Licence: This item is available under a Creative Commons Attribution Non Commercial Share Alike Licence (CC BY-NC-SA). Details of this licence are available here

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