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EXT1:EXT2 transfer GlcNAc to the heparan chain

Stable Identifier
R-HSA-2022851
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
Another GlcNAc addition extends the heparan chain
Locations in the PathwayBrowser
Summation

Exostosin1 and 2 (EXT1 and 2) are dual specificity enzymes which catalyze the addition of N acetylglucosamine (GlcNAc) and glucuronate (GlcA) to extend the GAG chain on the protein linker sequence. Heparan is synthesized once GlcNAc is transferred to this 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
glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity of EXT1:EXT2 [Golgi membrane]
Physical Entity
Activity
Orthologous Events