ENZYME entry: EC 2.4.1.224
| Accepted Name |
|---|
| Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-
acetylglucosaminyltransferase.
|
| Alternative Name(s) |
|---|
| Alpha-N-acetylglucosaminyltransferase II. |
| Glucuronyl-N-acetylglucosaminylproteoglycan alpha-1,4-N-
acetylglucosaminyltransferase. |
| Reaction catalysed |
|---|
| UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan <=> UDP + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan |
| Comment(s) |
|---|
- Involved in the biosynthesis of heparin and heparan sulfate.
- Some forms of the enzyme from human (particularly the enzyme complex
encoded by the EXT1 and EXT2 genes) act as bifunctional
glycosyltransferases, which also have the EC 2.4.1.225 activity
required for the synthesis of the heparan sulfate disaccharide
repeats.
- Other human forms of this enzyme (e.g. the product of the EXTL1 gene)
have only the 4-alpha-N-acetylglucosaminyltransferase activity.
- In Caenorhabditis elegans, the product of the rib-2 gene displays the
activities of this enzyme as well as EC 2.4.1.223.
|
| Cross-references |
|---|
| BRENDA | 2.4.1.224 |
| EC2PDB | 2.4.1.224 |
| ExplorEnz | 2.4.1.224 |
| PRIAM enzyme-specific profiles | 2.4.1.224 |
| KEGG Ligand Database for Enzyme Nomenclature | 2.4.1.224 |
| IUBMB Enzyme Nomenclature | 2.4.1.224 |
| IntEnz | 2.4.1.224 |
| MEDLINE | Find literature relating to 2.4.1.224 |
| MetaCyc | 2.4.1.224 |
| UniProtKB/Swiss-Prot |
| Q5IGR8, EXT1A_DANRE; | Q5IGR7, EXT1B_DANRE; | Q5IGR6, EXT1C_DANRE; |
| A5D7I4, EXT1_BOVIN; | Q9JK82, EXT1_CRIGR; | Q9V730, EXT1_DROME; |
| Q16394, EXT1_HUMAN; | P97464, EXT1_MOUSE; | A9X1C8, EXT1_PAPAN; |
| Q5RBC3, EXT1_PONAB; | O77783, EXT2_BOVIN; | O01705, EXT2_CAEEL; |
| Q9Y169, EXT2_DROME; | Q93063, EXT2_HUMAN; | P70428, EXT2_MOUSE; |
| Q92935, EXTL1_HUMAN; | Q9JKV7, EXTL1_MOUSE; |
|
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