EXT1:EXT2 transfers GlcA to heparan

Stable Identifier
R-HSA-2076392
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
Another GlcA addition extends the heparan chain
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).

Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity of EXT1:EXT2 [Golgi membrane]
Physical Entity
Activity
Orthologous Events