Reactome: A Curated Pathway Database

EXT1:EXT2 transfers GlcNAc to heparan

Stable Identifier
R-HSA-2022856
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
Summation

Exostosin 1 and 2 (EXT1 and 2) are dual specificity enzymes which catalyze the addition of N-acetylglucosamine (GlcNAc) and glucuronate (GlcA) to the GAG protein linker sequence. The first addition mediated by these enzymes is that of glucuronate after EXTL1 has added the first GlcNAc unit to the linkage sequence. EXT1 and 2 form a heterodimer which translocates to the Golgi apparatus from the ER membrane (McCormick et al. 2000). Defects in EXT1 or 2 cause the hereditary bone disorders multiple exostoses type 1 (MIM:133700) and 2 (MIM:133701) (Wuyts et al. 1998, Bernard et al. 2001).

Literature References
Participants
Participant Of
This entity is regulated by:
Title Physical Entity Activity
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity of EXT1:EXT2 [Golgi membrane] EXT1:EXT2 [Golgi membrane] N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity (0050509)
Orthologous Events