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Proteins/UNIPROT:Q16739
Participates in the initial step of the glucosylceramide-based glycosphingolipid/GSL synthetic pathway at the cytosolic surface of the Golgi. Catalyzes the transfer of glucose from UDP-glucose to ceramide to produce glucosylceramide/GlcCer (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine). GlcCer is the core component of glycosphingolipids/GSLs, amphipathic molecules consisting of a ceramide lipid moiety embedded in the outer leaflet of the membrane, linked to one of hundreds of different externally oriented oligosaccharide structures. Glycosphingolipids are essential components of membrane microdomains that mediate membrane trafficking and signal transduction, implicated in many fundamental cellular processes, including growth, differentiation, migration, morphogenesis, cell-to-cell and cell-to-matrix interactions (By similarity). They are required for instance in the proper development and functioning of the nervous system (By similarity). As an example of their role in signal transduction, they regulate the leptin receptor/LEPR in the leptin-mediated signaling pathway (By similarity). They also play an important role in the establishment of the skin barrier regulating keratinocyte differentiation and the proper assembly of the cornified envelope (By similarity). The biosynthesis of GSLs is also required for the proper intestinal endocytic uptake of nutritional lipids (By similarity). Catalyzes the synthesis of xylosylceramide/XylCer (such as beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine) using UDP-Xyl as xylose donor..
Function from UniProtKB (Q16739), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
263 statements
263 statements · 7 reference databases · Subject: UGCG · Sources: Bgee, gene ontology, Hetionet, Human-GEM, Reactome, Rhea, WikiPathways
5 reactions
an N-acylsphing-4-enine+MAM03108cD-glucosyl-N-acylsphingosine+proton+UDP
Catalysed by UGCG
Reaction pageLeft to rightBalance undeterminableEC 2.4.1.80cytosol
Human-GEM MAR00761CC BY 4.0Reference data
an N-acylsphing-4-enine+MAM03108rD-glucosyl-N-acylsphingosine+proton+UDP
Catalysed by UGCG
6 names
Resolves to: UGCG
Human-GEM MAR08248CC BY 4.0Reference data
an N-acylsphing-4-enine+UDP-alpha-D-glucosea beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine+UDP+hydron
Catalysed by UGCG
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:12088CC BY 4.0Reference data
UDP-alpha-D-xylose+an N-acylsphing-4-eninea beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine+UDP+hydron
Catalysed by UGCG
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:70243CC BY 4.0Reference data
N-(9Z-octadecenoyl)-sphing-4-enine+UDP-alpha-D-xylosebeta-D-xylosyl-(1<->1')-N-(9Z-octadecenoyl)-sphing-4-enine+UDP+hydron
Catalysed by UGCG
Reaction pageLeft to rightBalanced
Rhea RHEA:70247CC BY 4.0Reference data