Pflügers Archiv

, Volume 447, Issue 5, pp 768–775

Molecular physiology and pathology of the nucleotide sugar transporter family (SLC35)

The ABC of Solute carriers Guest Editor: Matthias A. Hediger

DOI: 10.1007/s00424-003-1093-0

Cite this article as:
Ishida, N. & Kawakita, M. Pflugers Arch - Eur J Physiol (2004) 447: 768. doi:10.1007/s00424-003-1093-0

Abstract

The solute carrier family SLC35 consists of at least 17 molecular species in humans. The family members so far characterized encode nucleotide sugar transporters localizing at the Golgi apparatus and/or the endoplasmic reticulum (ER). These transporters transport nucleotide sugars pooled in the cytosol into the lumen of these organelles, where most glycoconjugate synthesis occurs. Pathological analyses and developmental studies of small, multicellular organisms deficient in nucleotide sugar transporters have shown these transporters to be involved in tumour metastasis, cellular immunity, organogenesis and morphogenesis. Leukocyte adhesion deficiency type II (LAD II) or the congenital disorder of glycosylation type IIc (CDG IIc) are the sole human congenital disorders known to date that are caused by a defect of GDP-fucose transport. Along with LAD II, the possible involvement of nucleotide sugar transporters in disorders of connective tissues and muscles is also discussed.

Keywords

Cancer metastasis Congenital disorders of glycosylation Ehlers-Danlos syndrome Glycoconjugate Golgi apparatus Inclusion body myopathy Notch receptor Nucleotide sugar transporter 

Copyright information

© Springer-Verlag  2004

Authors and Affiliations

  1. 1.Department of Biochemical Cell ResearchTokyo Metropolitan Institute of Medical ScienceTokyoJapan
  2. 2.Department of Applied ChemistryKogakuin UniversityTokyoJapan

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