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Human deficiencies of fucosylation and sialylation affecting selectin ligands

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Abstract

Selectins are carbohydrate-binding adhesion molecules that are required for leukocyte trafficking to secondary lymphoid organs and to sites of infection. They interact with fucosylated and sialylated ligands bearing sialyl-Lewis X as a minimal carbohydrate structure. With this in mind, it should be expected that individuals with deficient fucosylation or sialylation show immunodeficiency. However, as this review shows, the picture appears to be more complex and more interesting. Although there are only few patients with such glycosylation defects, they have turned out to be very instructive for our understanding of the functions of fucosylation and sialylation in immunity, development and hemostasis.

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Acknowledgements

We would like to thank the patients and their parents for their patience with us, for providing blood samples and for their permission to publish the data.

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  1. Weatherall Institute of Molecular Medicine, MRC Human Immunology Unit, John Radcliffe Hospital, University of Oxford, Oxford, OX3 9DS, UK

    Kerstin Lühn

  2. Max Planck Institute for Molecular Biomedicine, Röntgenstr. 20, 48149, Münster, Germany

    Martin K. Wild

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  1. Kerstin Lühn
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Correspondence to Martin K. Wild.

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This article is published as part of the Special Issue on Glycosylation and Immunity [34:3].

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Lühn, K., Wild, M.K. Human deficiencies of fucosylation and sialylation affecting selectin ligands. Semin Immunopathol 34, 383–399 (2012). https://doi.org/10.1007/s00281-012-0304-1

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