Summary: Glycosphingolipids containing N-acetylglucosamine (paragloboside) or N-acetylgalactosamine (globoside). Globoside is the P antigen on erythrocytes and paragloboside is an intermediate in the biosynthesis of erythrocyte blood group ABH and P 1 glycosphingolipid antigens. The accumulation of globoside in tissue, due to a defect in hexosaminidases A and B, is the cause of Sandhoff disease.

Top Publications

  1. Meisen I, Friedrich A, Karch H, Witting U, Peter Katalinic J, Muthing J. Application of combined high-performance thin-layer chromatography immunostaining and nanoelectrospray ionization quadrupole time-of-flight tandem mass spectrometry to the structural characterization of high- and low-affinity binding ligands of Shiga. Rapid Commun Mass Spectrom. 2005;19:3659-65 pubmed
  2. Porubsky S, Speak A, Luckow B, Cerundolo V, Platt F, Grone H. Normal development and function of invariant natural killer T cells in mice with isoglobotrihexosylceramide (iGb3) deficiency. Proc Natl Acad Sci U S A. 2007;104:5977-82 pubmed
    ..Our results strongly suggest that iGb3 is unlikely to be an endogenous CD1d lipid ligand determining thymic iNKT selection. ..
  3. Paton A, Morona R, Paton J. Neutralization of Shiga toxins Stx1, Stx2c, and Stx2e by recombinant bacteria expressing mimics of globotriose and globotetraose. Infect Immun. 2001;69:1967-70 pubmed
    ..4% of the cytotoxicity in lysates of E. coli JM109 expressing cloned stx2e. These data suggest that either globotriose- or globotetraose-expressing constructs may be suitable for treatment and/or prevention of edema disease in pigs. ..
  4. Porubsky S, Speak A, Salio M, Jennemann R, Bonrouhi M, Zafarulla R, et al. Globosides but not isoglobosides can impact the development of invariant NKT cells and their interaction with dendritic cells. J Immunol. 2012;189:3007-17 pubmed publisher
    ..cells in ?-galactosidase A-deficient (?GalA(-/-)) mice (human Fabry disease), which accumulate isoglobosides and globosides. However, the presence and function of iGb3 in murine thymus remained controversial...
  5. Muthing J, Meisen I, Zhang W, Bielaszewska M, Mormann M, Bauerfeind R, et al. Promiscuous Shiga toxin 2e and its intimate relationship to Forssman. Glycobiology. 2012;22:849-62 pubmed publisher
  6. Godfrey D, McConville M, Pellicci D. Chewing the fat on natural killer T cell development. J Exp Med. 2006;203:2229-32 pubmed
    ..This suggests that lysosomal storage diseases (LSDs) in general, rather than one specific defect, can disrupt CD1d antigen presentation, leading to impaired development of NKT cells. ..
  7. Speak A, Salio M, Neville D, Fontaine J, Priestman D, Platt N, et al. Implications for invariant natural killer T cell ligands due to the restricted presence of isoglobotrihexosylceramide in mammals. Proc Natl Acad Sci U S A. 2007;104:5971-6 pubmed
    ..iGb3 is therefore unlikely to be a physiologically relevant iNKT cell-selecting ligand in mouse and humans. A detailed study is now warranted to better understand the nature of iNKT cell-selecting ligand(s) in vivo. ..
  8. Cheng L, Ueno A, Cho S, Im J, Golby S, Hou S, et al. Efficient activation of Valpha14 invariant NKT cells by foreign lipid antigen is associated with concurrent dendritic cell-specific self recognition. J Immunol. 2007;178:2755-62 pubmed
    ..Thus, CD1d-dependent potentiation by DCs may be crucial for iNKT cell-mediated immunity against infectious agents. ..
  9. Keusch J, Manzella S, Nyame K, Cummings R, Baenziger J. Expression cloning of a new member of the ABO blood group glycosyltransferases, iGb3 synthase, that directs the synthesis of isoglobo-glycosphingolipids. J Biol Chem. 2000;275:25308-14 pubmed
    ..As an indirect consequence of the expression cloning strategy used, we have been able to identify several potential glycolipid biosynthetic pathways where iGb(3) functions, including the globo- and isoglobo-series of glycolipids. ..

More Information


  1. Xia C, Zhou D, Liu C, Lou Y, Yao Q, Zhang W, et al. Thio-isoglobotrihexosylceramide, an agonist for activating invariant natural killer T cells. Org Lett. 2006;8:5493-6 pubmed
    ..The biological assay showed that S-iGb3 demonstrates a much higher increase as a stimulatory ligand toward invariant natural killer T (iNKT) cells as compared to iGb3. [structure: see text]. ..
  2. Xia C, Yao Q, Schumann J, Rossy E, Chen W, Zhu L, et al. Synthesis and biological evaluation of alpha-galactosylceramide (KRN7000) and isoglobotrihexosylceramide (iGb3). Bioorg Med Chem Lett. 2006;16:2195-9 pubmed
    ..Biological assays on the synthetic KRN7000 and iGb3 were performed using human and murine iNKT cell clones or hybridomas. ..
  3. Iwamura K, Furukawa K, Uchikawa M, Sojka B, Kojima Y, Wiels J, et al. The blood group P1 synthase gene is identical to the Gb3/CD77 synthase gene. A clue to the solution of the P1/P2/p puzzle. J Biol Chem. 2003;278:44429-38 pubmed
    ..Differences in the transcriptional regulation in erythrocytes might be a major factor determining P1/P2. ..
  4. Godfrey D, Pellicci D, Smyth M. Immunology. The elusive NKT cell antigen--is the search over?. Science. 2004;306:1687-9 pubmed
  5. Cooling L, Zhang D, Naides S, Koerner T. Glycosphingolipid expression in acute nonlymphocytic leukemia: common expression of shiga toxin and parvovirus B19 receptors on early myeloblasts. Blood. 2003;101:711-21 pubmed
    ..Expression of globotetraosylceramide, the parvovirus B19 receptor, on myeloblasts may also explain the association between B19 infection, aplastic anemia, and chronic neutropenia of childhood. ..
  6. Li Y, Teneberg S, Thapa P, Bendelac A, Levery S, Zhou D. Sensitive detection of isoglobo and globo series tetraglycosylceramides in human thymus by ion trap mass spectrometry. Glycobiology. 2008;18:158-65 pubmed
  7. Pina D, Johannes L, Castanho M. Shiga toxin B-subunit sequential binding to its natural receptor in lipid membranes. Biochim Biophys Acta. 2007;1768:628-36 pubmed
    ..Based on our observations, a model of the protein-membrane interaction is proposed and the STxB membrane partition and binding constants were calculated. ..
  8. Li Y, Zhou D, Xia C, Wang P, Levery S. Sensitive quantitation of isoglobotriaosylceramide in the presence of isobaric components using electrospray ionization-ion trap mass spectrometry. Glycobiology. 2008;18:166-76 pubmed
    ..This methodology may have wide implications for functional glycosphingolipidomics of immune cells and glycosphingolipid biomarker analysis. ..
  9. Zhou D, Mattner J, Cantu C, Schrantz N, Yin N, Gao Y, et al. Lysosomal glycosphingolipid recognition by NKT cells. Science. 2004;306:1786-9 pubmed
    ..We suggest that expression of iGb3 in peripheral tissues may be involved in controlling NKT cell responses to infections and malignancy and in autoimmunity. ..
  10. Schumann J, Facciotti F, Panza L, Michieletti M, Compostella F, Collmann A, et al. Differential alteration of lipid antigen presentation to NKT cells due to imbalances in lipid metabolism. Eur J Immunol. 2007;37:1431-41 pubmed
    ..Based on these results, the impact of lipid accumulation should be generally considered in the interpretation of immunological deficiencies found in mice suffering from lipid metabolic disorders. ..
  11. Schaade L, Kleines M, Walter R, Thomssen R, Ritter K. A membrane-located glycosphingolipid of monocyte/granulocyte lineage cells induces growth arrest and triggers the lytic viral cycle in Epstein-Barr virus genome-positive Burkitt lymphoma lines. Med Microbiol Immunol. 1999;188:23-9 pubmed
    ..Growth inhibition and termination of the viral latency suggest that IV3NeuAc-nLc4 present in monocyte/granulocyte lineage cells may be an effector of the natural defense against EBV persistency and transformation. ..
  12. Suchanowska A, Kaczmarek R, Duk M, Lukasiewicz J, Smolarek D, Majorczyk E, et al. A single point mutation in the gene encoding Gb3/CD77 synthase causes a rare inherited polyagglutination syndrome. J Biol Chem. 2012;287:38220-30 pubmed publisher
    ..Collectively, these results suggest that the C631G mutation alters the acceptor specificity of Gb3/CD77 synthase, rendering it able to catalyze synthesis of the Gal(?1-4)Gal and Gal(?1-4)GalNAc moieties. ..
  13. Sarkar A, Drouillard S, Rivet A, Perez S. Databases of Conformations and NMR Structures of Glycan Determinants. Glycobiology. 2015;25:1480-90 pubmed publisher
    ..core and lacto-series), sialylated oligosaccharides (Types 1 and 2), Lewis antigens, GPI-anchors and globosides. A complementary set of about 100 glycan determinants occurring in glycoproteins and glycosaminoglycans has also ..
  14. Ishitoya S, Yamamoto S, Mitsumori K, Ogawa O, Terai A. Non-secretor status is associated with female acute uncomplicated pyelonephritis. BJU Int. 2002;89:851-4 pubmed
    ..These results suggest that non-secretor status is associated with a genetic susceptibility to acute uncomplicated pyelonephritis, especially in premenopausal women. ..
  15. Kuijf M, Eurelings M, Tio Gillen A, Van Doorn P, van den Berg L, Hooijkaas H, et al. Detection of anti-MAG antibodies in polyneuropathy associated with IgM monoclonal gammopathy. Neurology. 2009;73:688-95 pubmed publisher
    ..In this study, the diagnostic accuracy of a new and more practical ELISA to detect these antibodies was validated...
  16. Kakuda S, Sato Y, Tonoyama Y, Oka S, Kawasaki T. Different acceptor specificities of two glucuronyltransferases involved in the biosynthesis of HNK-1 carbohydrate. Glycobiology. 2005;15:203-10 pubmed
    ..These lines of evidence indicate that these two enzymes have significantly different acceptor specificities, suggesting that they may synthesize functionally and structurally different HNK-1 carbohydrates in the nervous system. ..
  17. Basu M, Weng S, Tang H, Khan F, Rossi F, Basu S. Biosynthesis in vitro of neolactotetraosylceramide by a galactosyltransferase from mouse T-lymphoma: purification and kinetic studies; synthesis of neolacto and polylactosamine core. Glycoconj J. 1996;13:423-32 pubmed
  18. Vogel A, Furst W, Abo Hashish M, Lee Vaupel M, Conzelmann E, Sandhoff K. Identity of the activator proteins for the enzymatic hydrolysis of sulfatide, ganglioside GM1, and globotriaosylceramide. Arch Biochem Biophys. 1987;259:627-38 pubmed
    ..In contrast, the activator for ganglioside GM2 hydrolysis could clearly be separated from the other activities. Kinetic data were determined for the interaction of the sulfatide activator with the different glycolipids and hydrolases. ..
  19. Yamawaki M, Ariga T, Bigbee J, Ozawa H, Kawashima I, Tai T, et al. Generation and characterization of anti-sulfoglucuronosyl paragloboside monoclonal antibody NGR50 and its immunoreactivity with peripheral nerve. J Neurosci Res. 1996;44:586-93 pubmed
    ..Faint staining was also visible at the axolemmal-myelin interface; however, compact myelin was not stained. ..
  20. Pellicci D, Clarke A, Patel O, Mallevaey T, Beddoe T, Le Nours J, et al. Recognition of ?-linked self glycolipids mediated by natural killer T cell antigen receptors. Nat Immunol. 2011;12:827-33 pubmed publisher
    ..Thus, the NKT cell TCR molds the ?-linked self ligands to resemble the conformation of foreign ?-linked ligands, which shows that induced-fit molecular mimicry can underpin the self-reactivity of NKT cell TCRs to ?-linked antigens. ..
  21. Kijimoto Ochiai S, Tashiro A, Katagiri Y, Hatae T, Kobayashi S, Okuyama H. Forssman antigen expressed on lymph node cells of MRL/MpJ-lpr/lpr mice is of a glycoprotein nature. Microbiol Immunol. 1990;34:299-309 pubmed
    ..No significant difference of the SRBC hemolysis levels was observed between the sera from MRL/MpJ-lpr/lpr and its congenic MRL/MpJ-+/+ mice. ..
  22. Clark G, Gorbea C, Cummings R, Mattox S, Smith D. Decreased biosynthesis of Forssman glycolipid after retinoic acid-induced differentiation of mouse F9 teratocarcinoma cells. Lectin-affinity chromatography of the glycolipid-derived oligosaccharide. Carbohydr Res. 1991;213:155-68 pubmed
    ..These data demonstrated that F9 cells synthesize authentic Forssman glycolipid, and that its expression and the activity of Forssman synthase were decreased following induced cellular differentiation. ..
  23. Shimoda Y, Tajima Y, Nagase T, Harii K, Osumi N, Sanai Y. Cloning and expression of a novel galactoside beta1, 3-glucuronyltransferase involved in the biosynthesis of HNK-1 epitope. J Biol Chem. 1999;274:17115-22 pubmed
    ..Regions that expressed GlcAT-D and/or GlcAT-P were always HNK-1-positive, indicating that both GlcATs are involved in the synthesis of the HNK-1 epitope in vivo. ..
  24. Hellberg A, Westman J, Thuresson B, Olsson M. P1PK: the blood group system that changed its name and expanded. Immunohematology. 2013;29:25-33 pubmed
    ..Recently, it was shown that the same A4GALT-encoded galactosyltransferase synthesizes both the P1 and Pk antigens and that a polymorphism in a new exon in this gene predicts the P1 and P2 phenotypes. ..
  25. Ikeda J, Kohriyama T, Nakamura S. Elevation of serum soluble E-selectin and antisulfoglucuronyl paragloboside antibodies in amyotrophic lateral sclerosis. Eur J Neurol. 2000;7:541-7 pubmed
    ..3 +/- 21.1 ng/ml). Anti-SGPG antibodies may take part in the activation and/or injury of endothelial cells. The increased expression of E-selectin may be related to an immunological process in some ALS patients. ..
  26. Duk M, Singh S, Reinhold V, Krotkiewski H, Kurowska E, Lisowska E. Structures of unique globoside elongation products present in erythrocytes with a rare NOR phenotype. Glycobiology. 2007;17:304-12 pubmed
    ..These results indicate that polyagglutination in NOR subjects is due to unique erythrocyte glycolipids that are synthesized by sequential addition of Galalpha1,4 and GalNAcbeta1,3 to globoside. ..
  27. Ryu K, Lin S, Shao J, Song J, Chen M, Wang W, et al. Synthesis of complex carbohydrates and glyconjugates: enzymatic synthesis of globotetraose using alpha-1,3-N-acetylgalactosaminyltransferase LgtD from Haemophilus infuenzae strain Rd. Methods Mol Biol. 2005;310:93-105 pubmed
  28. Thakral B, Bhattacharya P, Agnihotri N, Sharma R, Marwaha N, Gopalan S. Acute hemolytic transfusion reaction by anti-P1 antibody in pregnancy. Am J Hematol. 2005;78:163-4 pubmed
  29. Yamamura T, Sakuishi K, Illes Z, Miyake S. Understanding the behavior of invariant NKT cells in autoimmune diseases. J Neuroimmunol. 2007;191:8-15 pubmed
    ..Understanding of how iNKT cells regulate autoimmunity should lead to a more sophisticated strategy for controlling autoimmune diseases. ..
  30. Yu E, Girardi E, Wang J, Zajonc D. Cutting edge: structural basis for the recognition of ?-linked glycolipid antigens by invariant NKT cells. J Immunol. 2011;187:2079-83 pubmed publisher
    ..Finally, the formation of novel contacts between the proximal and second sugar of iGb3 and CDR2? residues of the TCR suggests an expanded recognition logic that can possibly distinguish foreign Ags from self Ags. ..
  31. Drouet A, Caudie C, Vallat J, Ruel J, Felten D, Guilloton L, et al. [Polyneuropathy involving cranial nerves associated with monoclonal IgM antibodies with anti-MAG/SGPG/SGPLG/sulfatides activity]. Rev Neurol (Paris). 2006;162:760-6 pubmed
  32. Hood D, Cox A, Wakarchuk W, Schur M, Schweda E, Walsh S, et al. Genetic basis for expression of the major globotetraose-containing lipopolysaccharide from H. influenzae strain Rd (RM118). Glycobiology. 2001;11:957-67 pubmed
    ..This is the first genetic blueprint determined for H. influenzae LPS oligosaccharide biosynthesis, identifying genes involved in the addition of each glycose residue. ..
  33. Thomaidis T, Relle M, Golbas M, Brochhausen C, Galle P, Beck M, et al. Downregulation of alpha-galactosidase A upregulates CD77: functional impact for Fabry nephropathy. Kidney Int. 2009;75:399-407 pubmed publisher
  34. Orino K, Naiki M. Two kinds of P-fimbrial variants of uropathogenic Escherichia coli recognizing forssman glycosphingolipid. Microbiol Immunol. 1990;34:607-15 pubmed
    ..This adhesin was completely different from P adhesin and Forssman-like adhesin in recognition of the carbohydrate epitope. This adhesin, until now called a pseudotype of P fimbriae, was renamed Forssman adhesin. ..
  35. Rogers T, Paton J. Therapeutic strategies for Shiga toxin-producing Escherichia coli infections. Expert Rev Anti Infect Ther. 2009;7:683-6 pubmed publisher
    ..Accordingly, this report will focus on papers presented at the meeting that addressed development of alternative therapeutic strategies. ..
  36. He H, Nilsson C, Emmett M, Marshall A, Kroes R, Moskal J, et al. Glycomic and transcriptomic response of GSC11 glioblastoma stem cells to STAT3 phosphorylation inhibition and serum-induced differentiation. J Proteome Res. 2010;9:2098-108 pubmed publisher
    ..The synergies derived by the combination of glycomic, transcriptomic and phosphoproteomic data may aid our understanding of intracellular and cell-surface events associated with CSC differentiation. ..
  37. Katagiri Y, Sato B, Yamatoya K, Taki T, Goto Inoue N, Setou M, et al. GalNAc?1,3-linked paragloboside carries the epitope of a sperm maturation-related glycoprotein that is recognized by the monoclonal antibody MC121. Biochem Biophys Res Commun. 2011;406:326-31 pubmed publisher
    ..These results indicated that the epitope to which MC121 binds is present in a neolacto-series GSL, IV³GalNAc?-nLc?Cer² sequence. ..
  38. Skinner C, McMahon S, Rasooly R, Carter J, He X. Purification and characterization of Shiga toxin 2f, an immunologically unrelated subtype of Shiga toxin 2. PLoS ONE. 2013;8:e59760 pubmed publisher
    ..coli strain isolated from a feral pigeon. Information obtained in this study will be valuable for characterizing Stx2f and explaining the differences of Stx2a and Stx2f in host specificity and cytotoxicity. ..
  39. Randriantsoa M, Drouillard S, Breton C, Samain E. Synthesis of globopentaose using a novel beta1,3-galactosyltransferase activity of the Haemophilus influenzae beta1,3-N-acetylgalactosaminyltransferase LgtD. FEBS Lett. 2007;581:2652-6 pubmed
    ..In vitro assays confirmed that LgtD had both beta1,3-GalT and beta1,3-GalNAcT activities and showed that differences in the affinity for Gb3 and Gb4 explain the specific and exclusive formation of globopentaose. ..
  40. Tippett P, Andrews P, Knowles B, Solter D, Goodfellow P. Red cell antigens P (globoside) and Luke: identification by monoclonal antibodies defining the murine stage-specific embryonic antigens -3 and -4 (SSEA-3 and SSEA-4). Vox Sang. 1986;51:53-6 pubmed
    ..Using MC813-70, the frequencies of the 3 phenotypes LKE+, LKEw and LKE- in an English donor population are 0.914, 0.072 and 0.014, respectively. ..
  41. Yamaji T, Nishikawa K, Hanada K. Transmembrane BAX inhibitor motif containing (TMBIM) family proteins perturbs a trans-Golgi network enzyme, Gb3 synthase, and reduces Gb3 biosynthesis. J Biol Chem. 2010;285:35505-18 pubmed publisher
    ..Furthermore, GRINA-C was associated with Gb3 synthase. These observations may demonstrate a new type of posttranscriptional regulation of glycosyltransferases. ..
  42. Bergh A, Bhattacharyya S, Nilsson U. Synthesis of a globotetraose trimer. Carbohydr Res. 2002;337:947-9 pubmed
    ..The synthesis was readily monitored and purified using reversed phase HPLC. Unreacted squaric decyl ester globotetraoside was recovered rendering the method highly economical. ..
  43. Kronenberg M, Gapin L. Natural killer T cells: know thyself. Proc Natl Acad Sci U S A. 2007;104:5713-4 pubmed
  44. Nilsson U, Ray A, Magnusson G. Synthesis of the Forssman pentasaccharide and terminal tetra-, tri-, and di-saccharide fragments. Carbohydr Res. 1994;252:137-48 pubmed
    ..The anomeric TMSEt group of the Forssman pentasaccharide and terminal tetrasaccharide was removed with trifluoroacetic acid-dichloromethane, to give the corresponding hemiacetal sugars 4 and 6. ..
  45. Okada N, Yasuda T, Tsumita T, Okada H. Activation of the alternative complement pathway by natural antibody to glycolipids in guinea-pig serum. Immunology. 1983;50:75-84 pubmed
    ..This conclusion indicated that natural antibodies of the IgM type might play a role with ACP in host defence, especially in C4-deficient guinea-pigs where the classical complement pathway is impaired. ..
  46. Pourshafie M, Marklund B, Ohlson S. Binding interactions of Escherichia coli with globotetraosylceramide (globoside) using a surface plasmon resonance biosensor. J Microbiol Methods. 2004;58:313-20 pubmed
    ..coli interactions. This model may assist in screening for compounds that can inhibit the binding of uropathogenic E. coli to glycolipid ligands on target cells. ..
  47. Okuda T, Nakakita S, Nakayama K. Structural characterization and dynamics of globotetraosylceramide in vascular endothelial cells under TNF-alpha stimulation. Glycoconj J. 2010;27:287-96 pubmed publisher
  48. Betz J, Bauwens A, Kunsmann L, Bielaszewska M, Mormann M, Humpf H, et al. Uncommon membrane distribution of Shiga toxin glycosphingolipid receptors in toxin-sensitive human glomerular microvascular endothelial cells. Biol Chem. 2012;393:133-47 pubmed publisher
    ..Our finding opens new questions on the microdomain association of Stx receptors and the functional role of GSLs in the membrane assembly of GMVECs. ..
  49. Chou D, Henion T, Jungalwala F. Regulation of expression of sulfoglucuronyl carbohydrate (HNK-1), Amphoterin and RAGE in retinoic acid-differentiated P19 embryonal carcinoma cells. J Neurochem. 2003;86:917-31 pubmed
  50. Zhou D. The immunological function of iGb3. Curr Protein Pept Sci. 2006;7:325-33 pubmed
    ..New immunotherapy approaches might be explored by interfering with glycolipid metabolism or by directly supplementing rationally designed glycolipids. ..
  51. Fragaki K, Ait El Mkadem S, Chaussenot A, Gire C, Mengual R, Bonesso L, et al. Refractory epilepsy and mitochondrial dysfunction due to GM3 synthase deficiency. Eur J Hum Genet. 2013;21:528-34 pubmed publisher
    ..The accumulation of Gb3 and Gb4 globosides likely has a role in RC dysfunction and in the decrease of mitochondrial membrane potential leading to apoptosis,..
  52. Mori M, Sadahira Y, Kawasaki S, Hayashi T, Awai M. Macrophage heterogeneity in bone marrow culture in vitro. J Cell Sci. 1990;95 ( Pt 3):481-5 pubmed
    ..The data suggest that Forssman GSL positive macrophages, derived from resident bone marrow macrophages, play an important role in hematopoiesis and are clearly distinguished from small macrophages in vitro. ..
  53. Sato B, Katagiri Y, Miyado K, Okino N, Ito M, Akutsu H, et al. Lipid rafts enriched in monosialylGb5Cer carrying the stage-specific embryonic antigen-4 epitope are involved in development of mouse preimplantation embryos at cleavage stage. BMC Dev Biol. 2011;11:22 pubmed publisher
    ..These results indicate that MSGb5Cer-enriched raft membrane domains participate in cytokinesis in a close cooperation with the cortical actin network and the distribution of E-cadherin. ..
  54. Storck W, Meisen I, Gianmoena K, Pläger I, Kouzel I, Bielaszewska M, et al. Shiga toxin glycosphingolipid receptor expression and toxin susceptibility of human pancreatic ductal adenocarcinomas of differing origin and differentiation. Biol Chem. 2012;393:785-99 pubmed publisher
  55. Liang Y, Kuo H, Lin C, Chen Y, Yang B, Cheng Y, et al. Switching of the core structures of glycosphingolipids from globo- and lacto- to ganglio-series upon human embryonic stem cell differentiation. Proc Natl Acad Sci U S A. 2010;107:22564-9 pubmed publisher
    ..well-known hESC-specific markers stage-specific embryonic antigen 3 (SSEA-3) and SSEA-4, we identified several globosides and lacto-series GSLs, previously unrevealed in hESCs, including Gb(4)Cer, Lc(4)Cer, fucosyl Lc(4)Cer, Globo H, ..
  56. Rombach S, Aerts J, Poorthuis B, Groener J, Donker Koopman W, Hendriks E, et al. Long-term effect of antibodies against infused alpha-galactosidase A in Fabry disease on plasma and urinary (lyso)Gb3 reduction and treatment outcome. PLoS ONE. 2012;7:e47805 pubmed publisher
    ..Anti agalsidase ABs negatively influence globotriaosylceramide (Gb3) reduction. We investigated the impact of agalsidase AB on Gb3 and lysoGb3 and clinical outcome in Fabry patients on ERT...
  57. Kavaliauskiene S, Nymark C, Bergan J, Simm R, Sylvänne T, Simolin H, et al. Cell density-induced changes in lipid composition and intracellular trafficking. Cell Mol Life Sci. 2014;71:1097-116 pubmed publisher
    ..In conclusion, our data provide novel information on how lipid composition changes during cell growth in culture, and how these changes can modulate intracellular trafficking. ..
  58. Jaskowski T, Martins T, Litwin C, Hill H. Immunoglobulin (Ig) M antibody against myelin associated glycoprotein (MAG): A comparison of methods. J Clin Lab Anal. 2004;18:247-50 pubmed
    ..The authors recommend screening for MAG IgM in suspected patient sera by SGPG EIA or myelin IFA and utilizing these same methods to titer sera confirmed positive by WB. ..
  59. Li Y, Thapa P, Hawke D, Kondo Y, Furukawa K, Furukawa K, et al. Immunologic glycosphingolipidomics and NKT cell development in mouse thymus. J Proteome Res. 2009;8:2740-51 pubmed publisher
    ..Our results also provide early insights into cellular lipidomics studies, with a specific focus on the important immunological functions of glycosphingolipids. ..
  60. Johansson P, Nilsson J, Angstrom J, Miller Podraza H. Interaction of Helicobacter pylori with sialylated carbohydrates: the dependence on different parts of the binding trisaccharide Neu5Ac{alpha}3Gal{beta}4GlcNAc. Glycobiology. 2005;15:625-36 pubmed
    ..GlcNAc does influence binding, however, primarily serving as a guiding carrier for the binding epitope rather than being a part of the binding structure. ..
  61. Capasso M, Torrieri F, Di Muzio A, De Angelis M, Lugaresi A, Uncini A. Can electrophysiology differentiate polyneuropathy with anti-MAG/SGPG antibodies from chronic inflammatory demyelinating polyneuropathy?. Clin Neurophysiol. 2002;113:346-53 pubmed
    ..A disproportionate slowing of conduction in distal segments of motor nerves suggests the diagnosis of anti-MAG/SGPG neuropathy, although it is not pathognomonic. ..
  62. Alvarez L, del Pozo C, Trigueros M, Sanchez L, Albero M, López Menchero R, et al. [Enzyme replacement therapy in Fabry's disease]. Nefrologia. 2005;25:322-7 pubmed
    ..Six months after the end of the therapy, a reduction in the impairment of renal function is observed, and the classic manifestations of the disease are absent. ..
  63. Christiansen D, Milland J, Mouhtouris E, Vaughan H, Pellicci D, McConville M, et al. Humans lack iGb3 due to the absence of functional iGb3-synthase: implications for NKT cell development and transplantation. PLoS Biol. 2008;6:e172 pubmed publisher
    ..Furthermore, the absence of iGb3 in humans implies that it is another source of foreign Galalpha(1,3)Gal xenoantigen, with obvious significance in the field of xenotransplantation. ..
  64. Caudie C, Kaygisiz F, Jaquet P, Petiot P, Gonnaud P, Antoine J, et al. [Diagnostic value of autoantibodies to MAG by ELISA Bühlmann in 117 immune-mediated peripheral neuropathies associated with monoclonal IgM to SGPG/SGLPG]. Ann Biol Clin (Paris). 2006;64:353-9 pubmed
    ..It is also alpha good test to appreciate clinical improvement after Rituximab treatment. ..
  65. Bailly P, Piller F, Gillard B, Veyrières A, Marcus D, Cartron J. Biosynthesis of the blood group Pk and P1 antigens by human kidney microsomes. Carbohydr Res. 1992;228:277-87 pubmed
    ..The results suggested that the Pk and P1 glycolipids are synthesized by two distinct enzymes. ..
  66. Mishra R, Grzybek M, Niki T, Hirashima M, Simons K. Galectin-9 trafficking regulates apical-basal polarity in Madin-Darby canine kidney epithelial cells. Proc Natl Acad Sci U S A. 2010;107:17633-8 pubmed publisher
    ..Together our results suggest a model wherein such glycosphingolipid-galectin couples form a circuit between the Golgi apparatus and the cell surface that in an epithelial context facilitates the apical sorting of proteins and lipids. ..
  67. Westman J, Hellberg A, Peyrard T, Hustinx H, Thuresson B, Olsson M. P1/P2 genotyping of known and novel null alleles in the P1PK and GLOB histo-blood group systems. Transfusion. 2013;53:2928-39 pubmed publisher
    ..Furthermore, P(1) /P(2) genotyping predicted the P1 (k) versus P2 (k) phenotype in more than 90% of globoside-deficient samples. The number of GLOB-null alleles was increased by 50% and several P1PK-null alleles were identified. ..
  68. Kanai Y, Kawakami H, Takata K, Kurohmaru M, Hayashi Y, Nishida T, et al. Localization of Forssman glycolipid and GM1 ganglioside intracellularly and on the surface of germ cells during fetal testicular and ovarian development of mice. Histochemistry. 1990;94:561-8 pubmed
    ..c. (oocytes in the first meiotic prophase).(ABSTRACT TRUNCATED AT 250 WORDS) ..
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