EXTL3 dimer transfers GlcNAc to the GAG linker
UDP-GlcNAc + GlcA-Gal-Gal-Xyl-HS proteins → UDP + Heparan-PGs + H+
- Reactome
- R-HSA-9953259
- Compartment
- Golgi membrane · Golgi lumen
- Rhea
- RHEA:16222
Inputs
- UDP-GlcNAcCHEBI:57705, C17H25N3O17P2, Golgi lumen
- GlcA-Gal-Gal-Xyl-HS proteinsR-HSA-2076551, Golgi lumen
Outputs
- UDPCHEBI:58223, C9H11N2O12P2, Golgi lumen
- Heparan-PGsR-HSA-2076465, Golgi lumen
- H+CHEBI:15378, H, Golgi lumen
Catalysed by
- glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity of EXTL2,3 [Golgi membrane]EC 2.4.1.223
Literature
- Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha 1,4- N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/ heparin biosynthesis Proc Natl Acad Sci U S A, 2001
- The structure of EXTL3 helps to explain the different roles of bi-domain exostosins in heparan sulfate synthesis Nat Commun, 2022
- Mutations in EXTL3 Cause Neuro-immuno-skeletal Dysplasia Syndrome Am J Hum Genet, 2017
- EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay J Exp Med, 2017
- The tumor suppressor EXT-like gene EXTL2 encodes an alpha1, 4-N-acetylhexosaminyltransferase that transfers N-acetylgalactosamine and N-acetylglucosamine to the common glycosaminoglycan-protein linkage region. The key enzyme for the chain initiation of heparan sulfate J Biol Chem, 1999