Nucleotide sugar transporters of the Golgi apparatus: from basic science to diseases

Acc Chem Res. 2006 Nov;39(11):805-12. doi: 10.1021/ar0400239.


Approximately 80% of secreted and membrane proteins (40% of all proteins) of eukaryotes become covalently linked to sugars in the lumen of the Golgi apparatus, a cellular organelle that is part of the secretory system of all eukaryotes. The sugar donors are mostly nucleoside diphosphate sugars (nucleotide sugars) and must be translocated from the cytosol, their site of synthesis, across the Golgi apparatus membrane and into the lumen by specific transporters. These are hydrophobic, homodimeric proteins that span the membrane multiple times. Mutants of these proteins have developmental phenotypes including diseases in humans and cattle.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Biological Science Disciplines*
  • Biological Transport
  • Disease* / etiology
  • Golgi Apparatus / metabolism*
  • Humans
  • Membrane Transport Proteins* / chemistry
  • Membrane Transport Proteins* / genetics
  • Membrane Transport Proteins* / metabolism
  • Molecular Sequence Data
  • Mutation
  • Nucleoside Diphosphate Sugars / chemistry
  • Nucleoside Diphosphate Sugars / metabolism*
  • Substrate Specificity


  • Membrane Transport Proteins
  • Nucleoside Diphosphate Sugars