title: The exostosin family: proteins with many functions.
abstract: 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.
authors.affiliation: Department of Biochemistry, University of Cambridge, Cambridge, CB2 1QW, United Kingdom.
authors.forename: Marta
authors.initials: M
authors.lastname: Busse-Wicher
authors.affiliation: Wellcome Trust/Cancer Research UK, Gurdon Institute of Cancer and Developmental Biology, University of Cambridge, Cambridge CB2 1QN, United Kingdom.
authors.forename: Krzysztof B
authors.initials: KB
authors.lastname: Wicher
authors.affiliation: Department of Biomedicine, University of Bergen, 5009 Bergen, Norway. Electronic address: marion.kusche@biomed.uib.no.
authors.forename: Marion
authors.initials: M
authors.lastname: Kusche-Gullberg
mesh_terms: D000595:Amino Acid Sequence; D001483:Base Sequence; D005097:Exostoses, Multiple Hereditary / Q000235:genetics; D005109:Extracellular Matrix / Q000378:metabolism*; D016695:Glycosyltransferases / Q000378:metabolism*; D006497:Heparan Sulfate / Q000378:metabolism*; D006801:Humans; D008958:Models, Molecular; D008969:Molecular Sequence Data; D005810:Multigene Family / Q000502:physiology*; D017351:N-Acetylglucosaminyltransferases / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism / Q000502:physiology*; D010802:Phylogeny; D058105:Polymerization; D016415:Sequence Alignment; D017422:Sequence Analysis, DNA; D013045:Species Specificity; D000099058:Exostosin 1
chemical_list: D016695:Glycosyltransferases; D006497:Heparan Sulfate; D017351:N-Acetylglucosaminyltransferases; D000099058:Exostosin 1
keywords: EXT1; EXT2; EXTL; Glycosyltransferase activities; Heparan sulfate