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Information on EC - glucuronosyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase

for references in articles please use BRENDA:EC2.4.1.223
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IUBMB Comments
Enzyme involved in the initiation of heparin and heparan sulfate synthesis, transferring GlcNAc to the (GlcA-Gal-Gal-Xyl-)Ser core. Apparently products of both the human EXTL2 and EXTL3 genes can catalyse this reaction. In Caenorhabditis elegans, the product of the rib-2 gene displays this activity as well as that of EC, glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase. For explanation of the use of a superscript in the systematic name, see {iupac/2carb/37#372::2-Carb-37.2}.
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The enzyme appears in viruses and cellular organisms
extl2, alpha1,4-n-acetylglucosaminyltransferase, brother of tout-velu, exostosin-like 2, galntl-1, alpha-n-acetylglucosaminyltransferase i, more
UDP-N-acetyl-alpha-D-glucosamine + [protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine = UDP + [protein]-3-O-(alpha-D-GlcNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine
show the reaction diagram
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