The EXT1 subunit of the EXT1:EXT2 complex transfers a glucur...

created [InstanceEdit:9941068] Stephan, Ralf, 2025-03-07
dbId 9941339
displayName The EXT1 subunit of the EXT1:EXT2 complex transfers a glucur...
schemaClass Summation
text The EXT1 subunit of the EXT1:EXT2 complex transfers a glucuronyl (GlcA) moiety from UDP-GlcA to the growing heparan chain of HSPGs. 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. 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).
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