Proteins/UNIPROT:Q8N3Y3

LARGE2

ProteinUNIPROT:Q8N3Y3Xylosyl- and glucuronyltransferase LARGE2

Bifunctional glycosyltransferase with both alpha-1,3-xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the maturation of alpha-dystroglycan (DAG1) by glycosylation leading to DAG1 binding to laminin G-like domain-containing extracellular proteins with high affinity and in a phosphorylated-O-mannosyl trisaccharide dependent manner. Elongates the glucuronyl-beta-1,4-xylose-beta disaccharide primer structure by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide (By similarity). Supports the maturation of DAG1 more effectively than LARGE1. In addition, can modify both heparan sulfate (HS)- and chondroitin/dermatan sulfate (CS/DS)-proteoglycans (PGs), namely GPC4, with a glycosaminoglycan (GAG)-like polysaccharide composed of xylose and glucuronic acid to confer laminin binding (By similarity)..

Function from UniProtKB (Q8N3Y3), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Statements

12 statements

12 statements · 5 reference databases · Subject: LARGE2 · Sources: Hetionet, Reactome, Rhea, UniProt, WikiPathways

Order

ARHGDIG covaries with LARGE2ProteinHetionet hetionet:covaries:120071-398Inferred only
LRRC31 covaries with LARGE2ProteinHetionet hetionet:covaries:120071-79782Inferred only
B4GAT1 interacts with LARGE2ProteinHetionet hetionet:interacts:11041-120071Inferred only
LARGE2 participates in 11p11 2 copy number variation syndrome PathwayWikiPathways WP5348Inferred only
LARGE2 participates in DAG1 glycosylationsPathwayReactome R-HSA-8931838Inferred only
LARGE2 participates in Matriglycan biosynthesis on DAG1PathwayReactome R-HSA-9939291Inferred only
LARGE2 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
LARGE2 participates in O-linked glycosylationPathwayReactome R-HSA-5173105Inferred only
LARGE2 participates in Post-translational protein modificationPathwayReactome R-HSA-597592Inferred only
LARGE2 requires the cofactor Mn(2+)CompoundUniProt Q8N3Y3Inferred only
UDP-alpha-D-xylose is a substrate of LARGE2CompoundRhea RHEA:57336Inferred only
UDP-alpha-D-xylose is a substrate of LARGE2CompoundRhea RHEA:68368Inferred only

Reactions

3 reactions

3-O-[beta-D-GlcA-(1->3)-beta-D-Xyl-(1->4)-Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-(O-6-P-alpha-D-Man)]-Thr-[protein]+UDP-alpha-D-xylose3-O-[alpha-D-Xyl-(1->3)-beta-D-GlcA-(1->4)-beta-D-Xyl-(1->4)-Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-(O-6-P-alpha-D-Man)]-Thr-[protein]+UDP+hydron
Catalysed by LARGE1 or LARGE2
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:57336CC BY 4.0Reference data
3-O-{(1->[3)-alpha-D-Xyl-(1->3)-beta-D-GlcA-(1->](n)-4)-beta-D-Xyl-(1->4)-Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-O-6-P-alpha-D-Man}-L-Thr-[protein]+UDP-alpha-D-glucuronate

Other names

4 names

Resolves to: LARGE2

Also called: ; Beta-1,3-glucuronyltransferase LARGE2Glycosyltransferase-like 1BGYLTL1BLARGE xylosyl- and glucuronyltransferase 2

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