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Chemical Compound Review

GluCer     N-[(E,2S,3R)-3-hydroxy-1- [(2R,3R,4S,5S,6R)...

Synonyms: AC1O5192, glucosylceramide
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Disease relevance of glucosylceramide


High impact information on glucosylceramide


Chemical compound and disease context of glucosylceramide


Biological context of glucosylceramide


Anatomical context of glucosylceramide

  • We conclude that the fluorescent sphingomyelin and glucosylceramide were delivered from the Golgi complex to the plasma membrane where they accumulated in the external leaflet of the membrane bilayer [20].
  • Internalization and sorting of a fluorescent analogue of glucosylceramide to the Golgi apparatus of human skin fibroblasts: utilization of endocytic and nonendocytic transport mechanisms [21].
  • When C5-DMB-Cer was used to label living cells, this property allowed us to differentiate membranes containing high concentrations of the fluorescent lipid and its metabolites (the corresponding analogues of sphingomyelin and glucosylceramide) from other regions of the cell where smaller amounts of the probe were present [22].
  • In dramatic contrast with MDCK cells, the Fischer rat thyroid (FRT) cell line targeted both gD1-DAF and GlcCer basolaterally [23].
  • The transbilayer movement of GlcCer and NFA-GalCer in the Golgi complex is an absolute requirement for higher glycosphingolipid biosynthesis and for the cell surface expression of these monohexosyl sphingolipids [24].

Associations of glucosylceramide with other chemical compounds


Gene context of glucosylceramide


Analytical, diagnostic and therapeutic context of glucosylceramide


  1. Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage. Mizukami, H., Mi, Y., Wada, R., Kono, M., Yamashita, T., Liu, Y., Werth, N., Sandhoff, R., Sandhoff, K., Proia, R.L. J. Clin. Invest. (2002) [Pubmed]
  2. The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms. Steet, R.A., Chung, S., Wustman, B., Powe, A., Do, H., Kornfeld, S.A. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  3. Plasma glucosylceramide deficiency as potential risk factor for venous thrombosis and modulator of anticoagulant protein C pathway. Deguchi, H., Fernández, J.A., Pabinger, I., Heit, J.A., Griffin, J.H. Blood (2001) [Pubmed]
  4. Inhibition of melanoma tumor growth by a novel inhibitor of glucosylceramide synthase. Weiss, M., Hettmer, S., Smith, P., Ladisch, S. Cancer Res. (2003) [Pubmed]
  5. Demonstration of direct glycosylation of nondegradable glucosylceramide analogs in cultured cells. Schwarzmann, G., Hofmann, P., Pütz, U., Albrecht, B. J. Biol. Chem. (1995) [Pubmed]
  6. An unusual glucocerebroside in the crustacean nervous system. Shimomura, K., Hanjura, S., Ki, P.F., Kishimoto, Y. Science (1983) [Pubmed]
  7. Status of blood group carbohydrate chains in ontogenesis and in oncogenesis. Watanabe, K., Hakomori, S.I. J. Exp. Med. (1976) [Pubmed]
  8. Cryptococcal virulence: beyond the usual suspects. Mitchell, A.P. J. Clin. Invest. (2006) [Pubmed]
  9. Defective TNF-alpha-mediated hepatocellular apoptosis and liver damage in acidic sphingomyelinase knockout mice. García-Ruiz, C., Colell, A., Marí, M., Morales, A., Calvo, M., Enrich, C., Fernández-Checa, J.C. J. Clin. Invest. (2003) [Pubmed]
  10. Agents that reverse multidrug resistance, tamoxifen, verapamil, and cyclosporin A, block glycosphingolipid metabolism by inhibiting ceramide glycosylation in human cancer cells. Lavie, Y., Cao, H., Volner, A., Lucci, A., Han, T.Y., Geffen, V., Giuliano, A.E., Cabot, M.C. J. Biol. Chem. (1997) [Pubmed]
  11. Cytoprotective effect of glucosylceramide synthase inhibition against daunorubicin-induced apoptosis in human leukemic cell lines. Grazide, S., Terrisse, A.D., Lerouge, S., Laurent, G., Jaffrézou, J.P. J. Biol. Chem. (2004) [Pubmed]
  12. Cryptococcus neoformans, Candida albicans, and other fungi bind specifically to the glycosphingolipid lactosylceramide (Gal beta 1-4Glc beta 1-1Cer), a possible adhesion receptor for yeasts. Jimenez-Lucho, V., Ginsburg, V., Krivan, H.C. Infect. Immun. (1990) [Pubmed]
  13. Thrombophilic factors are not the leading cause of thrombosis in Behçet's disease. Leiba, M., Seligsohn, U., Sidi, Y., Harats, D., Sela, B.A., Griffin, J.H., Livneh, A., Rosenberg, N., Gelernter, I., Gur, H., Ehrenfeld, M. Ann. Rheum. Dis. (2004) [Pubmed]
  14. Glycoprotein storage in Gaucher disease: lectin histochemistry and biochemical studies. DeGasperi, R., Alroy, J., Richard, R., Goyal, V., Orgad, U., Lee, R.E., Warren, C.D. Lab. Invest. (1990) [Pubmed]
  15. Processing of epidermal glucosylceramides is required for optimal mammalian cutaneous permeability barrier function. Holleran, W.M., Takagi, Y., Menon, G.K., Legler, G., Feingold, K.R., Elias, P.M. J. Clin. Invest. (1993) [Pubmed]
  16. Expression cloning of a cDNA for human ceramide glucosyltransferase that catalyzes the first glycosylation step of glycosphingolipid synthesis. Ichikawa, S., Sakiyama, H., Suzuki, G., Hidari, K.I., Hirabayashi, Y. Proc. Natl. Acad. Sci. U.S.A. (1996) [Pubmed]
  17. Reversible infertility in male mice after oral administration of alkylated imino sugars: a nonhormonal approach to male contraception. van der Spoel, A.C., Jeyakumar, M., Butters, T.D., Charlton, H.M., Moore, H.D., Dwek, R.A., Platt, F.M. Proc. Natl. Acad. Sci. U.S.A. (2002) [Pubmed]
  18. Salvage of glucosylceramide by recycling after internalization along the pathway of receptor-mediated endocytosis. Kok, J.W., Eskelinen, S., Hoekstra, K., Hoekstra, D. Proc. Natl. Acad. Sci. U.S.A. (1989) [Pubmed]
  19. Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure. Liu, Y., Suzuki, K., Reed, J.D., Grinberg, A., Westphal, H., Hoffmann, A., Döring, T., Sandhoff, K., Proia, R.L. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  20. Sorting of sphingolipids in epithelial (Madin-Darby canine kidney) cells. van Meer, G., Stelzer, E.H., Wijnaendts-van-Resandt, R.W., Simons, K. J. Cell Biol. (1987) [Pubmed]
  21. Internalization and sorting of a fluorescent analogue of glucosylceramide to the Golgi apparatus of human skin fibroblasts: utilization of endocytic and nonendocytic transport mechanisms. Martin, O.C., Pagano, R.E. J. Cell Biol. (1994) [Pubmed]
  22. A novel fluorescent ceramide analogue for studying membrane traffic in animal cells: accumulation at the Golgi apparatus results in altered spectral properties of the sphingolipid precursor. Pagano, R.E., Martin, O.C., Kang, H.C., Haugland, R.P. J. Cell Biol. (1991) [Pubmed]
  23. VIP21/caveolin, glycosphingolipid clusters and the sorting of glycosylphosphatidylinositol-anchored proteins in epithelial cells. Zurzolo, C., van't Hof, W., van Meer, G., Rodriguez-Boulan, E. EMBO J. (1994) [Pubmed]
  24. Topology of sphingolipid galactosyltransferases in ER and Golgi: transbilayer movement of monohexosyl sphingolipids is required for higher glycosphingolipid biosynthesis. Burger, K.N., van der Bijl, P., van Meer, G. J. Cell Biol. (1996) [Pubmed]
  25. Consequences of beta-glucocerebrosidase deficiency in epidermis. Ultrastructure and permeability barrier alterations in Gaucher disease. Holleran, W.M., Ginns, E.I., Menon, G.K., Grundmann, J.U., Fartasch, M., McKinney, C.E., Elias, P.M., Sidransky, E. J. Clin. Invest. (1994) [Pubmed]
  26. Assignment of the gene for human sphingolipid activator protein-2 (SAP-2) to chromosome 10. Fujibayashi, S., Kao, F.T., Jones, C., Morse, H., Law, M., Wenger, D.A. Am. J. Hum. Genet. (1985) [Pubmed]
  27. Glycosphingolipid expression in acute nonlymphocytic leukemia: common expression of shiga toxin and parvovirus B19 receptors on early myeloblasts. Cooling, L.L., Zhang, d.e. .S., Naides, S.J., Koerner, T.A. Blood (2003) [Pubmed]
  28. Regulation of glycosphingolipid metabolism in liver during the acute phase response. Memon, R.A., Holleran, W.M., Uchida, Y., Moser, A.H., Ichikawa, S., Hirabayashi, Y., Grunfeld, C., Feingold, K.R. J. Biol. Chem. (1999) [Pubmed]
  29. Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth. Jennemann, R., Sandhoff, R., Wang, S., Kiss, E., Gretz, N., Zuliani, C., Martin-Villalba, A., Jäger, R., Schorle, H., Kenzelmann, M., Bonrouhi, M., Wiegandt, H., Gröne, H.J. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  30. Src kinase mediates the regulation of phospholipase C-gamma activity by glycosphingolipids. Shu, L., Shayman, J.A. J. Biol. Chem. (2003) [Pubmed]
  31. Gaucher disease mouse models: point mutations at the acid beta-glucosidase locus combined with low-level prosaposin expression lead to disease variants. Sun, Y., Quinn, B., Witte, D.P., Grabowski, G.A. J. Lipid Res. (2005) [Pubmed]
  32. MRP1 and glucosylceramide are coordinately over expressed and enriched in rafts during multidrug resistance acquisition in colon cancer cells. Klappe, K., Hinrichs, J.W., Kroesen, B.J., Sietsma, H., Kok, J.W. Int. J. Cancer (2004) [Pubmed]
  33. Reduction of globotriaosylceramide in Fabry disease mice by substrate deprivation. Abe, A., Gregory, S., Lee, L., Killen, P.D., Brady, R.O., Kulkarni, A., Shayman, J.A. J. Clin. Invest. (2000) [Pubmed]
  34. Effective cell and gene therapy in a murine model of Gaucher disease. Enquist, I.B., Nilsson, E., Ooka, A., Månsson, J.E., Olsson, K., Ehinger, M., Brady, R.O., Richter, J., Karlsson, S. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  35. Localization of ceramide and glucosylceramide in human epidermis by immunogold electron microscopy. Vielhaber, G., Pfeiffer, S., Brade, L., Lindner, B., Goldmann, T., Vollmer, E., Hintze, U., Wittern, K.P., Wepf, R. J. Invest. Dermatol. (2001) [Pubmed]
  36. An improved technique for separation of neutral glycosphingolipids by high-performance liquid chromatography. Suzuki, A., Kundu, S.K., Marcus, D.M. J. Lipid Res. (1980) [Pubmed]
  37. Long-term follow-up of the first successful bone marrow transplantation in Gaucher disease. Ringdén, O., Groth, C.G., Erikson, A., Bäckman, L., Granqvist, S., Månsson, J.E., Svennerholm, L. Transplantation (1988) [Pubmed]
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