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GDP-Fucose Transporter 1 (SLC35C1)

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Handbook of Glycosyltransferases and Related Genes

Abstract

GDP-fucose is used by fucosyltransferases in both the Golgi and endoplasmic reticulum (ER). As it is synthesized in the cytoplasm, transport into the lumen of the secretory pathway organelles is required. The first indication that GDP-fucose, like other nucleotide sugars, is transported by a carrier-mediated process was obtained by Sommers and Hirschberg (1982). The gene encoding the GDP-fucose transporter was identified independently by two groups using complementation cloning in patient-derived cells (Lübke et al. 2001; Lühn et al. 2001). These patients were suffering from leukocyte adhesion deficiency II (LAD II), also known as congenital disorder of glycosylation (CDG) type IIc. LAD II patients were originally described as having a deficiency of the fucosylated glycan Sialyl-Lewis X (Etzioni et al. 1992; Etzioni 1994; Phillips et al. 1995). The lack of fucose likely prevents interaction of the endothelial adhesion molecules E- and P-selectin with glycoproteins present on leukocytes. The latter are then unable to migrate through the endothelial layer to reach the sites of inflammation (Wild et al. 2002). In addition to the immunological problems, LAD II patients present characteristic symptoms of CDG patients such as severe mental and growth retardation (Etzioni 2010).

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Authors and Affiliations

  1. Institute for Cellular Chemistry, Hannover Medical School, Build. J3, Level 4, Room 1290, Carl-Neubergstrasse 1, 30625, Hannover, Germany

    Dr. Hans Bakker, Dr. Angel Ashikov, Dr. Francoise H. Routier & Dr. Rita Gerardy-Schahn

Authors
  1. Dr. Hans Bakker
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  2. Dr. Angel Ashikov
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  3. Dr. Francoise H. Routier
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  4. Dr. Rita Gerardy-Schahn
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Corresponding author

Correspondence to Hans Bakker .

Editor information

Editors and Affiliations

  1. RIKEN-Max-Planck Joint Research Center for Systems Chemical Biology, RIKEN, Wako, Saitama, Japan

    Naoyuki Taniguchi

  2. Department of Biochemistry, Kochi University, Medical School, Nankoku, Kochi, Japan

    Koichi Honke

  3. Sanford Burnham Medical Research Institute, La Jolla, California, USA

    Minoru Fukuda

  4. Research Center for Medical Glycoscience (RCMG), National Institute of Advanced Industrial Science and Technology (AIST), Tsukuba, Ibaraki, Japan

    Hisashi Narimatsu

  5. RIKEN-Max-Planck Joint Research Center for Systems Chemical Biology, RIKEN, Wako, Saitama, Japan

    Yoshiki Yamaguchi

  6. Institute of Biological Chemistry, Academia Sinica, Taipei, Taiwan

    Takashi Angata

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© 2014 Springer Japan

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Bakker, H., Ashikov, A., Routier, F.H., Gerardy-Schahn, R. (2014). GDP-Fucose Transporter 1 (SLC35C1). In: Taniguchi, N., Honke, K., Fukuda, M., Narimatsu, H., Yamaguchi, Y., Angata, T. (eds) Handbook of Glycosyltransferases and Related Genes. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54240-7_38

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