The exostosin family: proteins with many functions

Matrix Biol. 2014 Apr:35:25-33. doi: 10.1016/j.matbio.2013.10.001. Epub 2013 Oct 12.


Heparan sulfates are complex sulfated molecules found in abundance at cell surfaces and in the extracellular matrix. They bind to and influence the activity of a variety of molecules like growth factors, proteases and morphogens and are thus involved in various cell-cell and cell-matrix interactions. The mammalian EXT proteins have glycosyltransferase activities relevant for HS chain polymerization, however their exact role in this process is still confusing. In this review, we summarize current knowledge about the biochemical activities and some proposed functions of the members of the EXT protein family and their roles in human disease.

Keywords: EXT1; EXT2; EXTL; Glycosyltransferase activities; Heparan sulfate.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Amino Acid Sequence
  • Base Sequence
  • Exostoses, Multiple Hereditary / genetics
  • Extracellular Matrix / metabolism*
  • Glycosyltransferases / metabolism*
  • Heparitin Sulfate / metabolism*
  • Humans
  • Models, Molecular*
  • Molecular Sequence Data
  • Multigene Family / physiology*
  • N-Acetylglucosaminyltransferases / deficiency*
  • N-Acetylglucosaminyltransferases / genetics
  • N-Acetylglucosaminyltransferases / metabolism
  • N-Acetylglucosaminyltransferases / physiology*
  • Phylogeny
  • Polymerization
  • Sequence Alignment
  • Sequence Analysis, DNA
  • Species Specificity


  • Heparitin Sulfate
  • Glycosyltransferases
  • N-Acetylglucosaminyltransferases
  • exostosin-1