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Proteins/UNIPROT:Q93063
Glycosyltransferase forming with EXT1 the heterodimeric heparan sulfate polymerase which catalyzes the elongation of the heparan sulfate glycan backbone. Glycan backbone extension consists in the alternating transfer of (1->4)-beta-D-GlcA and (1->4)-alpha-D-GlcNAc residues from their respective UDP-sugar donors. Both EXT1 and EXT2 are required for the full activity of the polymerase since EXT1 bears the N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity within the complex while EXT2 carries the glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity. Heparan sulfate proteoglycans are ubiquitous components of the extracellular matrix and play an important role in tissue homeostasis and signaling..
Function from UniProtKB (Q93063), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
3 rows
91 statements
91 statements · 7 reference databases · Subject: EXT2 · Sources: Bgee, Hetionet, Human-GEM, Reactome, Rhea, UniProt, WikiPathways
9 reactions
MAM02081g+UDP-alpha-D-glucuronateproton+MAM02088g+UDP
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.224, 2.4.1.225Golgi apparatus
Human-GEM MAR07208CC BY 4.0Reference data
MAM02088g+CHEBI:16264proton+MAM02089g+UDP
4 names
Resolves to: EXT2
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.223, 2.4.1.224, 2.4.1.225Golgi apparatus
Human-GEM MAR07209CC BY 4.0Reference data
MAM02089g+UDP-alpha-D-glucuronateproton+MAM02090g+UDP
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.224, 2.4.1.225Golgi apparatus
Human-GEM MAR07210CC BY 4.0Reference data
MAM02090g+CHEBI:16264proton+MAM02091g+UDP
Catalysed by EXT1 & EXT2 or EXTL3 or EXTL1
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.223, 2.4.1.224, 2.4.1.225Golgi apparatus
Human-GEM MAR07211CC BY 4.0Reference data
MAM02091g+UDP-alpha-D-glucuronateproton+MAM02092g+UDP
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.224, 2.4.1.225Golgi apparatus
Human-GEM MAR07212CC BY 4.0Reference data
MAM02092g+CHEBI:16264proton+MAM02093g+UDP
Catalysed by EXT1 & EXT2 or EXTL3 or EXTL1
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.223, 2.4.1.224, 2.4.1.225Golgi apparatus
Human-GEM MAR07213CC BY 4.0Reference data
MAM02093g+UDP-alpha-D-glucuronateproton+MAM02094g+UDP
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.224, 2.4.1.225Golgi apparatus
Human-GEM MAR07214CC BY 4.0Reference data
MAM02094g+CHEBI:16264proton+MAM02095g+UDP
Catalysed by EXT1 & EXT2 or EXTL3 or EXTL1
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.223, 2.4.1.224, 2.4.1.225Golgi apparatus
Human-GEM MAR07215CC BY 4.0Reference data
3-O-{[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein]+UDP-N-acetyl-alpha-D-glucosamine3-O-{alpha-D-GlcNAc-[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein]+UDP+hydron
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:16213CC BY 4.0Reference data