EXT1:EXT2 transfers GlcA to heparan
UDP-GlcA + Heparan-PGs → UDP + Heparan(1)-PGs + H+
- Reactome
- R-HSA-2022856
- Compartment
- Golgi membrane · Golgi lumen
- Rhea
- RHEA:20909
Inputs
- UDP-GlcACHEBI:58052, C15H19N2O18P2, Golgi lumen
- Heparan-PGsR-HSA-2076465, Golgi lumen
Outputs
- UDPCHEBI:58223, C9H11N2O12P2, Golgi lumen
- Heparan(1)-PGsR-HSA-2076416, Golgi lumen
- H+CHEBI:15378, H, Golgi lumen
Catalysed by
- N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity of EXT1:EXT2 [Golgi membrane]EC 2.4.1.225
Literature
- 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
- 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