UGCG

Glycosphingolipids (GSLs) are a group of membrane components that contain lipid and sugar moieties. They are present in essentially all animal cells and are believed to have important roles in various cellular processes. UDP-glucose ceramide glucosyltransferase catalyzes the first glycosylation step in glycosphingolipid biosynthesis. The product, glucosylceramide, is the core structure of more than 300 GSLs. UGCG is widely expressed and transciption is upregulated during keratinocyte differentiation. [provided by RefSeq]

Full Name

UDP-glucose ceramide glucosyltransferase

Function

Participates in the initial step of the glucosylceramide-based glycosphingolipid/GSL synthetic pathway at the cytosolic surface of the Golgi (PubMed:8643456, PubMed:1532799).
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) (PubMed:1532799, PubMed:8643456).
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 (PubMed:8643456).
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 (PubMed:33361282).

Biological Process

Biological Process cell differentiation Source:UniProtKB
Biological Process cornified envelope assembly Source:UniProtKB
Biological Process epidermis development Source:ProtInc1 Publication
Biological Process establishment of skin barrier Source:UniProtKB
Biological Process glucosylceramide biosynthetic process Source:UniProtKB1 Publication
Biological Process glycosphingolipid metabolic process Source:Reactome
Biological Process intestinal lipid absorption Source:UniProtKB
Biological Process keratinocyte differentiation Source:UniProtKB
Biological Process leptin-mediated signaling pathway Source:UniProtKB
Biological Process neuron development Source:UniProtKB
Biological Process protein lipidation Source:UniProtKB
Biological Process regulation of signal transduction Source:UniProtKB

Cellular Location

Golgi apparatus membrane

Topology

Lumenal: 1-10
Helical: 11-32
Cytoplasmic: 33-195
Helical: 196-215
Lumenal: 216-287
Helical: 288-304
Cytoplasmic: 305-309
Helical: 310-328
Lumenal: 329-348
Helical: 349-369
Cytoplasmic: 370-394