Ceramide glucosyltransferase
Also known as: UGCG
Function
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.
Classification
- Family (Pfam)
- PF13506 Glyco_transf_21
- InterPro
- Ceramide_glucosylTrfase, Nucleotide-diphossugar_trans
- Functional cluster
- Oxidoreductases, Catalases & Peroxidases
Gene Ontology · 15
- GO:0000139 Golgi membrane
- GO:0016020 membrane
- GO:0008120 ceramide glucosyltransferase activity
- GO:0030154 cell differentiation
- GO:1903575 cornified envelope assembly
- GO:0008544 epidermis development
- GO:0061436 establishment of skin barrier
- GO:0006679 glucosylceramide biosynthetic process
- GO:0006688 glycosphingolipid biosynthetic process
- GO:0098856 intestinal lipid absorption
- GO:0030216 keratinocyte differentiation
- GO:0033210 leptin-mediated signaling pathway
- GO:0048666 neuron development
- GO:0006497 protein lipidation
- GO:0009966 regulation of signal transduction
Drugs targeting this protein · 4
- MIGLUSTAT inhibitor
- LUCERASTAT inhibitor
- VENGLUSTAT inhibitor
- ELIGLUSTAT TARTRATE inhibitor
Related proteins · sequence + function similarity
- Ceramide glucosyltransferase 1.00
- Ceramide glucosyltransferase 1.00
- Ceramide glucosyltransferase 0.99
- Ceramide glucosyltransferase-B 0.99
- Ceramide glucosyltransferase-A 0.98
- Ceramide glucosyltransferase 2 0.82
- Beta-1,4-mannosyltransferase bre-3 0.81
- Beta-1,4-mannosyltransferase bre-3 0.80
- Ceramide glucosyltransferase 3 0.80
- Glycolipid beta-1,4-mannosyltransferase egh 0.79
- Hyaluronan synthase 1 0.77
- Hyaluronan synthase 1 0.76
Co-cited proteins · studied together in the literature
- Ceramide glucosyltransferase 1 shared papers
- Reticulon-1 1 shared papers
- Ceramide glucosyltransferase 1 shared papers
- Cytosolic beta-glucosidase 1 shared papers
- Lysosomal acid glucosylceramidase 1 shared papers
Literature · 9 cited papers
- Human glucocerebrosidase mediates formation of xylosyl-cholesterol by beta-xylosidase and transxylosidase reactions. J. Lipid Res. · 2021
- The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
- DNA sequence and analysis of human chromosome 9. Nature · 2004
- Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat. Genet. · 2004
- Glucosylceramide synthase and its functional interaction with RTN-1C regulate chemotherapeutic-induced apoptosis in neuroepithelioma cells. Cancer Res. · 2003
- Up-regulation of glucosylceramide synthase expression and activity during human keratinocyte differentiation. J. Biol. Chem. · 1998
- PMID 8901638 Proc. Natl. Acad. Sci. U.S.A. · 1996
- Expression cloning of a cDNA for human ceramide glucosyltransferase that catalyzes the first glycosylation step of glycosphingolipid synthesis. Proc. Natl. Acad. Sci. U.S.A. · 1996
- Glucosylceramide is synthesized at the cytosolic surface of various Golgi subfractions. J. Cell Biol. · 1992