EXT1:EXT2 transfers GlcNAc to the heparan chain
UDP-GlcNAc + Heparan(1)-PGs → UDP + Heparan(2)-PGs + H+
- Reactome
- R-HSA-2022851
- Compartment
- Golgi membrane · Golgi lumen
- Rhea
- RHEA:16214
Inputs
- UDP-GlcNAcCHEBI:57705, C17H25N3O17P2, Golgi lumen
- Heparan(1)-PGsR-HSA-2076416, Golgi lumen
Outputs
- UDPCHEBI:58223, C9H11N2O12P2, Golgi lumen
- Heparan(2)-PGsR-HSA-2076412, Golgi lumen
- H+CHEBI:15378, H, Golgi lumen
Catalysed by
- glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity of EXT1:EXT2 [Golgi membrane]EC 2.4.1.224
Literature
- Diminished levels of the putative tumor suppressor proteins EXT1 and EXT2 in exostosis chondrocytes Cell Motil Cytoskeleton, 2001
- Mutations in the EXT1 and EXT2 genes in hereditary multiple exostoses Am J Hum Genet, 1998
- The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate Proc Natl Acad Sci U S A, 2000