Gene Symbol: Ugcg
Description: UDP-glucose ceramide glucosyltransferase
Alias: AU043821, C80537, Epcs21, GlcT-1, Ugcgl, ceramide glucosyltransferase, GCS, UDP-glucose:N-acylsphingosine D-glucosyltransferase, ectoplacental cone, invasive trophoblast giant cells, extraembryonic ectoderm and chorion sequence 21, glucosylceramide synthase
Species: mouse
Products:     Ugcg

Top Publications

  1. Jennemann R, Sandhoff R, Wang S, Kiss E, Gretz N, Zuliani C, et al. Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth. Proc Natl Acad Sci U S A. 2005;102:12459-64 pubmed
    ..UDP-glucose:ceramide glucosyltransferase (Ugcg) catalyzes the initial step of glycosphingolipid (GSL) biosynthesis...
  2. Stettner P, Bourgeois S, Marsching C, Traykova Brauch M, Porubsky S, Nordström V, et al. Sulfatides are required for renal adaptation to chronic metabolic acidosis. Proc Natl Acad Sci U S A. 2013;110:9998-10003 pubmed publisher
    ..This study points to a seminal role of sulfatides in renal ammonium handling, urinary acidification, and acid-base homeostasis. ..
  3. Yamashita T, Allende M, Kalkofen D, Werth N, Sandhoff K, Proia R. Conditional LoxP-flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis. Genesis. 2005;43:175-80 pubmed
    ..b>Glucosylceramide synthase--encoded by the Ugcg gene--controls the first committed step in the major pathway of glycosphingolipid ..
  4. Chatterjee S, Alsaeedi N, Hou J, Bandaru V, Wu L, Halushka M, et al. Use of a glycolipid inhibitor to ameliorate renal cancer in a mouse model. PLoS ONE. 2013;8:e63726 pubmed publisher
    ..Mice fed D- threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol (D-PDMP), an inhibitor of glucosylceramide synthase and lactosylceramide synthase (LCS: ?-1,4-GalT-V), showed marked reduction in tumor volume...
  5. Rovina P, Graf C, Bornancin F. Modulation of ceramide metabolism in mouse primary macrophages. Biochem Biophys Res Commun. 2010;399:150-4 pubmed publisher
    Ceramide kinase (CERK) produces the bioactive lipid ceramide 1-phosphate (C1P) and is, together with glucosylceramide synthase (GCS) and sphingomyelin synthases (SMS-1 and -2), a key regulator of ceramide metabolism...
  6. Watanabe S, Endo S, Oshima E, Hoshi T, Higashi H, Yamada K, et al. Glycosphingolipid synthesis in cerebellar Purkinje neurons: roles in myelin formation and axonal homeostasis. Glia. 2010;58:1197-207 pubmed publisher
    ..Glucosylceramide (GlcCer) synthase, which is encoded by the Ugcg gene, is the key enzyme driving the synthesis of most neuronal GSLs...
  7. Amen N, Mathow D, Rabionet M, Sandhoff R, Langbein L, Gretz N, et al. Differentiation of epidermal keratinocytes is dependent on glucosylceramide:ceramide processing. Hum Mol Genet. 2013;22:4164-79 pubmed publisher
    ..Tamoxifen (TAM)-induced deletion of the endogenous GlcCer-synthesizing enzyme UDP-glucose:ceramide glucosyltransferase (UGCG) in keratin K14-positive cells resulted in epidermal GlcCer depletion...
  8. Saadat L, Dupree J, Kilkus J, Han X, Traka M, Proia R, et al. Absence of oligodendroglial glucosylceramide synthesis does not result in CNS myelin abnormalities or alter the dysmyelinating phenotype of CGT-deficient mice. Glia. 2010;58:391-8 pubmed publisher
    ..central nervous system (CNS), mice were generated that lack oligodendroglial expression of UDP-glucose ceramide glucosyltransferase (encoded by Ugcg)...
  9. Chavez J, Siddique M, Wang S, Ching J, Shayman J, Summers S. Ceramides and glucosylceramides are independent antagonists of insulin signaling. J Biol Chem. 2014;289:723-34 pubmed publisher
    ..inhibitors of enzymes required for ceramide synthesis enhance insulin signaling, but those targeting glucosylceramide synthase have no effect...

More Information


  1. Jennemann R, Sandhoff R, Wiegandt H, Grone H. [Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth]. Verh Dtsch Ges Pathol. 2006;90:193-202 pubmed
    ..UDP-glucose: ceramide glucosyl-transferase (Ugcg) catalyzes the initial step of glycosphingolipid (GSL) biosynthesis...
  2. Porubsky S, Federico G, Muthing J, Jennemann R, Gretz N, Büttner S, et al. Direct acute tubular damage contributes to Shigatoxin-mediated kidney failure. J Pathol. 2014;234:120-33 pubmed publisher
    ..Specifically in young adults, an excellent outcome can be achieved by supportive therapy only. ..
  3. Yamashita T, Wada R, Proia R. Early developmental expression of the gene encoding glucosylceramide synthase, the enzyme controlling the first committed step of glycosphingolipid synthesis. Biochim Biophys Acta. 2002;1573:236-40 pubmed
    ..b>Glucosylceramide synthase converts ceramide to glucosylceramide, a core structure of the vast majority of GSLs...
  4. Mizukami H, Mi Y, Wada R, Kono M, Yamashita T, Liu Y, et al. Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage. J Clin Invest. 2002;109:1215-21 pubmed
    ..However, the mutant mice do not accumulate large amounts of glucosylceramide or exhibit classic Gaucher cells in tissues. ..
  5. Turáková K, Pavlíková L, Messingerová L, Lakatoš B, Breier A, Sulová Z. Reduced UDP-glucose Levels Are Associated with P-glycoprotein Over-expression in L1210 Cells and Limit Glucosylceramide Synthase Activity. Anticancer Res. 2015;35:2627-34 pubmed
    ..Several lines of evidence indicate that the expression and increased function of both Pgp and glucosylceramide synthase (GCS, an enzyme responsible for ceramide pathway de-activation in the regulation of apoptosis ..
  6. Yamashita T, Wada R, Sasaki T, Deng C, Bierfreund U, Sandhoff K, et al. A vital role for glycosphingolipid synthesis during development and differentiation. Proc Natl Acad Sci U S A. 1999;96:9142-7 pubmed eliminating the major synthesis pathway of GSLs through targeted disruption of the Ugcg gene encoding glucosylceramide synthase. In the absence of GSL synthesis, embryogenesis proceeded well into gastrulation with differentiation ..
  7. Herzer S, Meldner S, Gröne H, Nordström V. Fasting-Induced Lipolysis and Hypothalamic Insulin Signaling Are Regulated by Neuronal Glucosylceramide Synthase. Diabetes. 2015;64:3363-76 pubmed publisher
    ..Lipid microdomains contain large quantities of cholesterol and glycosphingolipids, including glucosylceramide synthase (GCS) (gene Ugcg)-derived gangliosides...
  8. Jennemann R, Rothermel U, Wang S, Sandhoff R, Kaden S, Out R, et al. Hepatic glycosphingolipid deficiency and liver function in mice. Hepatology. 2010;51:1799-809 pubmed publisher
    ..acting inhibitors for GSL biosynthesis have been used to inhibit the enzyme Ugcg (UDP-glucose:ceramide glucosyltransferase), catalyzing the first step of the glucosylceramide-based GSL-synthesis pathway...
  9. Ashe K, Budman E, Bangari D, Siegel C, Nietupski J, Wang B, et al. Efficacy of Enzyme and Substrate Reduction Therapy with a Novel Antagonist of Glucosylceramide Synthase for Fabry Disease. Mol Med. 2015;21:389-99 pubmed publisher
    ..of recombinant ?-Gal A, the merits of substrate reduction therapy (SRT) by selectively inhibiting glucosylceramide synthase (GCS) were examined...
  10. von Gerichten J, Schlosser K, Lamprecht D, Morace I, Eckhardt M, Wachten D, et al. Diastereomer-specific quantification of bioactive hexosylceramides from bacteria and mammals. J Lipid Res. 2017;58:1247-1258 pubmed publisher
    ..Finally, this method is shown to separate corresponding hexosylsphingosine standards, promoting its applicability in further investigations. ..
  11. Nordström V, Willershäuser M, Herzer S, Rozman J, Von Bohlen Und Halbach O, Meldner S, et al. Neuronal expression of glucosylceramide synthase in central nervous system regulates body weight and energy homeostasis. PLoS Biol. 2013;11:e1001506 pubmed publisher
    ..of metabolic signals requires neuronal expression of glucosylceramide synthase (GCS; UDP-glucose:ceramide glucosyltransferase)...
  12. Jennemann R, Kaden S, Sandhoff R, Nordström V, Wang S, Volz M, et al. Glycosphingolipids are essential for intestinal endocytic function. J Biol Chem. 2012;287:32598-616 pubmed
    ..The enzyme UDP-glucose ceramide glucosyltransferase (Ugcg) catalyzes the initial step of GSL biosynthesis...
  13. Pei B, Speak A, Shepherd D, Butters T, Cerundolo V, Platt F, et al. Diverse endogenous antigens for mouse NKT cells: self-antigens that are not glycosphingolipids. J Immunol. 2011;186:1348-60 pubmed publisher
    ..Our data indicate that there may be more than one type of self-Ag for iNKT cells, that the self-Ags comparing mouse and human may not be conserved, and that the search to identify these molecules should not be confined to GSLs. ..
  14. Ichikawa S, Ozawa K, Hirabayashi Y. Molecular cloning and expression of mouse ceramide glucosyltransferase. Biochem Mol Biol Int. 1998;44:1193-202 pubmed
    b>Ceramide glucosyltransferase (EC catalyzes the first glycosylation step of glycosphingolipid (GSL) synthesis, the transfer of glucose from UDP-Glucose to hydrophobic ceramide and generate glucosylceramide (GlcCer)...
  15. Xie W, Dolzhanskaya N, LaFauci G, Dobkin C, Denman R. Tissue and developmental regulation of fragile X mental retardation 1 exon 12 and 15 isoforms. Neurobiol Dis. 2009;35:52-62 pubmed publisher
    ..Altogether, the spatio-temporal plasticity of FMR1 mRNA is consistent with complex RNA processing that is mis-regulated in fragile X syndrome. ..
  16. Jennemann R, Sandhoff R, Langbein L, Kaden S, Rothermel U, Gallala H, et al. Integrity and barrier function of the epidermis critically depend on glucosylceramide synthesis. J Biol Chem. 2007;282:3083-94 pubmed ceramide metabolism to maintain epidermal barrier function, a mouse with an epidermis-specific glucosylceramide synthase (Ugcg) deficiency has been generated...
  17. Takagi Y, Nakagawa H, Matsuo N, Nomura T, Takizawa M, Imokawa G. Biosynthesis of acylceramide in murine epidermis: characterization by inhibition of glucosylation and deglucosylation, and by substrate specificity. J Invest Dermatol. 2004;122:722-9 pubmed
  18. Chen C, Kim M, Soo Lyu M, Kozak C, Schwartz R, Flomerfelt F. Characterization of the mouse gene, human promoter and human cDNA of TSCOT reveals strong interspecies homology. Biochim Biophys Acta. 2000;1493:159-69 pubmed
  19. Ichikawa S, Ozawa K, Hirabayashi Y. Assignment1 of a UDP-glucose:ceramide glucosyltransferase gene (Ugcg) to mouse chromosome band 4B3 by in situ hybridization. Cytogenet Cell Genet. 1998;83:14-5 pubmed
  20. Ichikawa S, Ozawa K, Hirabayashi Y. Molecular cloning and characterization of the mouse ceramide glucosyltransferase gene. Biochem Biophys Res Commun. 1998;253:707-11 pubmed
    b>Ceramide glucosyltransferase (glucosylceramide synthase, GlcT-1, EC catalyzes the first step in glycosphingolipid synthesis, the transfer of glucose from UDP-glucose to ceramide...