ID 2.4.1.224 DE glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- DE acetylglucosaminyltransferase. AN alpha-N-acetylglucosaminyltransferase II. AN glucuronyl-N-acetylglucosaminylproteoglycan alpha-1,4-N- AN acetylglucosaminyltransferase. CA 3-O-{[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)-beta-D-GlcA- CA (1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] CA + UDP-N-acetyl-alpha-D-glucosamine = 3-O-{alpha-D-GlcNAc-[(1->4)-beta-D- CA GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal- CA (1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] + H(+) + UDP. CC -!- Involved in the biosynthesis of heparin and heparan sulfate. CC -!- Some forms of the enzyme from human (particularly the enzyme complex CC encoded by the EXT1 and EXT2 genes) act as bifunctional CC glycosyltransferases, which also have the EC 2.4.1.225 activity CC required for the synthesis of the heparan sulfate disaccharide CC repeats. CC -!- Other human forms of this enzyme (e.g. the product of the EXTL1 gene) CC have only the 4-alpha-N-acetylglucosaminyltransferase activity. CC -!- In Caenorhabditis elegans, the product of the rib-2 gene displays the CC activities of this enzyme as well as EC 2.4.1.223. DR Q5IGR8, EXT1A_DANRE; Q5IGR7, EXT1B_DANRE; Q5IGR6, EXT1C_DANRE; DR Q9V730, EXT1_DROME ; O77783, EXT2_BOVIN ; O01705, EXT2_CAEEL ; DR Q9Y169, EXT2_DROME ; Q93063, EXT2_HUMAN ; P70428, EXT2_MOUSE ; DR Q92935, EXTL1_HUMAN; Q9JKV7, EXTL1_MOUSE; //