neutral glycosphingolipids


Summary: A subclass of GLYCOSPHINGOLIPIDS containing one or more sugars within their head group connected directly to a ceramide moiety. They consist of monoglycosyl-, and oligoglycosylsphingoids and monoglycosyl- and oligoglycosylceramides.

Top Publications

  1. Inagaki M, Saito T, Miyamoto T, Higuchi R. Isolation and structure of hematoside-type ganglioside from the starfish Linckia laevigata. Chem Pharm Bull (Tokyo). 2009;57:204-6 pubmed
    ..The ceramide moiety was composed of heterogeneous 2-hydroxy fatty acid and phytosphingosine units. This is the first report on the isolation and structure elucidation of naked hematoside-type ganglioside from echinoderms. ..
  2. Zarei M, Kirsch S, Muthing J, Bindila L, Peter Katalinic J. Automated normal phase nano high performance liquid chromatography/matrix assisted laser desorption/ionization mass spectrometry for analysis of neutral and acidic glycosphingolipids. Anal Bioanal Chem. 2008;391:289-97 pubmed publisher
    ..The method is demonstrated to be highly sensitive, reaching the low femtomole level of detection of individual GSL species and is highlighted as a versatile analytical tool for glycolipidomic studies. [figure: see text] ..
  3. Tani Y, Funatsu T, Ashida H, Ito M, Itonori S, Sugita M, et al. Novel neogala-series glycosphingolipids with terminal mannose and glucose residues from Hirsutella rhossiliensis, an aureobasidin A-resistant ascomycete fungus. Glycobiology. 2010;20:433-41 pubmed publisher
    ..In contrast, the ceramides of Glcbeta1-Cer contained d18:2- and d19:2-sphingoids. These findings indicate the presence of a novel biosynthetic pathway of neogala-series GSLs in fungi. ..
  4. Suzuki A, Miyazaki M, Matsuda J, Yoneshige A. High-performance thin-layer chromatography/mass spectrometry for the analysis of neutral glycosphingolipids. Biochim Biophys Acta. 2011;1811:861-74 pubmed publisher
    This mini-review summarizes the protocol we have developed for the analysis of neutral glycosphingolipids (GSLs) by high-performance thin layer chromatography (HPTLC)-mass spectrometry (MS)...
  5. Wichit S, Jittmittraphap A, Hidari K, Thaisomboonsuk B, Petmitr S, Ubol S, et al. Dengue virus type 2 recognizes the carbohydrate moiety of neutral glycosphingolipids in mammalian and mosquito cells. Microbiol Immunol. 2011;55:135-40 pubmed publisher
    ..The immunochemical detection and structural analysis of carbohydrates demonstrated that the neutral glycosphingolipids, L-3 (GlcNAc?1-3Man?1-4Glc?1-1'Cer) in AP-61 cells, and nLc(4) Cer (Gal?1-4GlcNAc?1-3Gal?1-4Glc?1-1'Cer) ..
  6. Nakamura K, Suzuki Y, Goto Inoue N, Yoshida Noro C, Suzuki A. Structural characterization of neutral glycosphingolipids by thin-layer chromatography coupled to matrix-assisted laser desorption/ionization quadrupole ion trap time-of-flight MS/MS. Anal Chem. 2006;78:5736-43 pubmed
    Rapid and convenient structural analysis of neutral glycosphingolipids (GSLs) was achieved by direct coupling of thin-layer chromatography (TLC) to matrix-assisted laser desorption/ionization quadrupole ion trap time-of-flight (MALDI-QIT-..
  7. Sugita M, Inagaki F, Naito H, Hori T. Studies on glycosphingolipids in larvae of the green-bottle fly, Lucilia caesar: two neutral glycosphingolipids having large straight oligosaccharide chains with eight and nine sugars. J Biochem. 1990;107:899-903 pubmed
    Two neutral glycosphingolipids having large straight oligosaccharide chains with eight and nine sugars, provisionally named COS and CNS, were isolated and purified from larvae of the green-bottle fly, Lucilia caesar, as the only two ..
  8. Tanaka K, Yamada M, Tamiya Koizumi K, Kannagi R, Aoyama T, Hara A, et al. Systematic analyses of free ceramide species and ceramide species comprising neutral glycosphingolipids by MALDI-TOF MS with high-energy CID. Glycoconj J. 2011;28:67-87 pubmed publisher
  9. Kojima H, Shimizu T, Sugita M, Itonori S, Fujita N, Ito M. Biochemical studies on sphingolipids of Artemia franciscana: novel neutral glycosphingolipids. J Lipid Res. 2011;52:308-17 pubmed publisher
    b>Neutral glycosphingolipids containing one to six sugars in their oligosaccharide chains have been isolated from cysts of the brine shrimp Artemia franciscana...

More Information


  1. Schweppe C, Hoffmann P, Nofer J, Pohlentz G, Mormann M, Karch H, et al. Neutral glycosphingolipids in human blood: a precise mass spectrometry analysis with special reference to lipoprotein-associated Shiga toxin receptors. J Lipid Res. 2010;51:2282-94 pubmed publisher
    ..are potential carriers of Stx through the circulation, we investigated human lipoprotein-associated neutral glycosphingolipids (GSLs) with emphasis on high (globotriaosylceramide) and low (globotetraosylceramide) affinity Stx-..
  2. Itonori S, Hashimoto K, Nakagawa M, Harada M, Suzuki T, Kojima H, et al. Structural analysis of neutral glycosphingolipids from the silkworm Bombyx mori and the difference in ceramide composition between larvae and pupae. J Biochem. 2017;: pubmed publisher
    ..MALDI-TOF MS analysis revealed that larval GSLs contained four types of ceramide species, whereas pupal GSLs contained only two types...
  3. Ozelsancak R, Uyar B. p.R301X Mutation and Variable Phenotypic Appearance of Fabry Disease. Am J Case Rep. 2016;17:315-9 pubmed
    Fabry disease is an X-linked disorder. Due to deficiency of the enzyme a-galactosidase A, neutral glycosphingolipids (primarily globotriaosylceramide) progressively accumulate within lysosomes of cells in various organ systems, resulting ..
  4. Ferraz M, Marques A, Gaspar P, Mirzaian M, van Roomen C, Ottenhoff R, et al. Lyso-glycosphingolipid abnormalities in different murine models of lysosomal storage disorders. Mol Genet Metab. 2016;117:186-93 pubmed publisher
    ..of Niemann Pick type C (Npc1nih and Npc1nmf164), revealing significant tissue elevation of several neutral glycosphingolipids and concomitant increased plasma glucosylsphingosine...
  5. Calixto R, Rollin Pinheiro R, da Silva M, Liporagi Lopes L, Vieira J, Sassaki G, et al. Structural analysis of glucosylceramides (GlcCer) from species of the Pseudallescheria/Scedosporium complex. Fungal Biol. 2016;120:166-72 pubmed publisher
    Glucosylceramides (GlcCer) are the main neutral glycosphingolipids expressed in fungal cells...
  6. Nguyen Dinh Cat A, Escoubet B, Agrapart V, Griol Charhbili V, Schoeb T, Feng W, et al. Cardiomyopathy and response to enzyme replacement therapy in a male mouse model for Fabry disease. PLoS ONE. 2012;7:e33743 pubmed publisher an X-linked disorder of glycosphingolipid metabolism that results in progressive accumulation of neutral glycosphingolipids, (predominately globotriaosylceramide; GL-3) in lysosomes, as well as other cellular compartments and ..
  7. Noël A, Ingrand S, Barrier L. Ganglioside and related-sphingolipid profiles are altered in a cellular model of Alzheimer's disease. Biochimie. 2017;137:158-164 pubmed publisher
    ..In this study, gangliosides and related-sphingolipids (ceramides, neutral glycosphingolipids and sphingomyelins) were analyzed in neuroglioma (H4) cells expressing the Swedish mutation of the human ..
  8. Kenoth R, Zou X, Simanshu D, Pike H, Malinina L, Patel D, et al. Functional evaluation of tryptophans in glycolipid binding and membrane interaction by HET-C2, a fungal glycolipid transfer protein. Biochim Biophys Acta Biomembr. 2018;1860:1069-1076 pubmed publisher
    ..that uses an evolutionarily-modified GLTP-fold to achieve more focused transfer specificity for simple neutral glycosphingolipids than mammalian GLTPs...
  9. Kanda T, Ariga T, Yamawaki M, Pal S, Katoh Semba R, Yu R. Effect of nerve growth factor and forskolin on glycosyltransferase activities and expression of a globo-series glycosphingolipid in PC12D pheochromocytoma cells. J Neurochem. 1995;64:810-7 pubmed
  10. Grange P, Erickson A, Levery S, Francis D. Identification of an intestinal neutral glycosphingolipid as a phenotype-specific receptor for the K88ad fimbrial adhesin of Escherichia coli. Infect Immun. 1999;67:165-72 pubmed
    ..Based on these studies, IGLad appears to be nLc4Cer. ..
  11. Cardoso C, Paviani E, Cruz L, Guma F, Borojevic R, Guaragna R. Effect of pentoxifylline on arachidonic acid metabolism, neutral lipid synthesis and accumulation during induction of the lipocyte phenotype by retinol in murine hepatic stellate cell. Mol Cell Biochem. 2003;254:37-46 pubmed
    ..In conclusion, PTF treatment generated an excess of free AA. We propose that retinol counteracts the action of PTF on the AA release and PGs production, even though both drugs stimulated the lipocyte induction in the HSC. ..
  12. Zancada L, Sánchez Juanes F, Alonso J, Hueso P. Neutral glycosphingolipid content of ovine milk. J Dairy Sci. 2010;93:19-26 pubmed publisher
    ..coli strains. These compounds could be used as an alternative and available source to supplement infant or bovine formulas with a view to preventing bacterial infections. ..
  13. Colombo I, Sottocornola E, Moretti S, Meloni M, Pippia P, Berra B. Modifications of glycosphingolipid profile and synthesis in normal rat fibroblasts and in syngeneic neoplastic cells at different subculture stages. Biochim Biophys Acta. 2000;1485:214-24 pubmed
  14. Farwanah H, Wirtz J, Kolter T, Raith K, Neubert R, Sandhoff K. Normal phase liquid chromatography coupled to quadrupole time of flight atmospheric pressure chemical ionization mass spectrometry for separation, detection and mass spectrometric profiling of neutral sphingolipids and cholesterol. J Chromatogr B Analyt Technol Biomed Life Sci. 2009;877:2976-82 pubmed publisher
    ..In addition, it can be of interest for research on disorders related to impairments of sphingolipid metabolism. ..
  15. Atshaves B, Jefferson J, McIntosh A, Gallegos A, McCann B, Landrock K, et al. Effect of sterol carrier protein-2 expression on sphingolipid distribution in plasma membrane lipid rafts/caveolae. Lipids. 2007;42:871-84 pubmed multiple sphingolipid species including ceramides, acidic glycosphingolipids (ganglioside GM1); neutral glycosphingolipids (monohexosides, dihexosides, globosides), and sphingomyelin as compared to non-raft domains...
  16. Maunula S, Björkqvist Y, Slotte J, Ramstedt B. Differences in the domain forming properties of N-palmitoylated neutral glycosphingolipids in bilayer membranes. Biochim Biophys Acta. 2007;1768:336-45 pubmed
    ..Our results show that the head group structures of neutral glycosphingolipids markedly affect their domain forming properties in bilayers both with and without cholesterol...
  17. Mihara T, Ueda A, Hirayama M, Takeuchi T, Yoshida S, Naito K, et al. Detection of new anti-neutral glycosphingolipids antibodies and their effects on Trk neurotrophin receptors. FEBS Lett. 2006;580:4991-5 pubmed
    We screened sera from patients with various neurological disorders for the presence of anti-neutral glycosphingolipids antibodies and only found them in sera from relapsing polychondritis with limbic encephalitis patients...
  18. Bodennec J, Pelled D, Futerman A. Aminopropyl solid phase extraction and 2 D TLC of neutral glycosphingolipids and neutral lysoglycosphingolipids. J Lipid Res. 2003;44:218-26 pubmed
    ..followed by multiple chromatography steps, yielding relatively low recoveries of n-lyso-GSLs and neutral glycosphingolipids (n-GSLs)...
  19. Loberto N, Prioni S, Bettiga A, Chigorno V, Prinetti A, Sonnino S. The membrane environment of endogenous cellular prion protein in primary rat cerebellar neurons. J Neurochem. 2005;95:771-83 pubmed
    ..All main sphingolipids, including sphingomyelin, neutral glycosphingolipids and gangliosides, were similarly enriched in the prion protein-rich membrane domains...
  20. Uemura A, Watarai S, Kushi Y, Kasama T, Ohnishi Y, Kodama H. Analysis of neutral glycosphingolipids from Trypanosoma brucei. Vet Parasitol. 2006;140:264-72 pubmed
    b>Neutral glycosphingolipids (GSLs) were isolated from Trypanosoma brucei and analyzed by thin-layer chromatography (TLC), TLC/secondary ion mass spectrometry (TLC/SIMS), and liposome immune lysis assay (LILA)...
  21. Ardail D, Popa I, Alcantara K, Pons A, Zanetta J, Louisot P, et al. Occurrence of ceramides and neutral glycolipids with unusual long-chain base composition in purified rat liver mitochondria. FEBS Lett. 2001;488:160-4 pubmed
    ..In contrast, 3-ketosphinganine was the only base of glucosylceramide and lactosylceramide of inner membranes, whereas d18:1 sphingosine was the major long-chain base of glucosylceramide of outer membranes. ..
  22. Zuolo M, Toledo M, Nogueira H, Straus A, Takahashi H. Identification of GM3 as a marker of therapy-resistant periradicular lesions. J Endod. 2001;27:107-9 pubmed
    ..In contrast lesion tissues express GM3, which is conspicuously absent in normal tissues. ..
  23. Kojima H, Tohsato Y, Kabayama K, Itonori S, Ito M. Biochemical studies on sphingolipids of Artemia franciscana: complex neutral glycosphingolipids. Glycoconj J. 2013;30:257-68 pubmed publisher
    ..CDeS, the longest GSL of this species, was found in the highest amount, which indicates that CDeS may be functionally important. ..
  24. Deguchi H, Fernandez J, Griffin J. Neutral glycosphingolipid-dependent inactivation of coagulation factor Va by activated protein C and protein S. J Biol Chem. 2002;277:8861-5 pubmed
    To test whether neutral glycosphingolipids can serve as anticoagulant cofactors, the effects of incorporation of neutral glycosphingolipids into phospholipid vesicles on anticoagulant and procoagulant reactions were studied...
  25. Arigi E, Singh S, Kahlili A, Winter H, Goldstein I, Levery S. Characterization of neutral and acidic glycosphingolipids from the lectin-producing mushroom, Polyporus squamosus. Glycobiology. 2007;17:754-66 pubmed
    ..Of these GSLs, Ps-2 could potentially interact with P. squamosus lectin, and further investigations will focus on determining the binding affinity, if any, of the lectin for the GIPCs isolated from this fungus. ..
  26. Hermansson M, Käkelä R, Berghäll M, Lehesjoki A, Somerharju P, Lahtinen U. Mass spectrometric analysis reveals changes in phospholipid, neutral sphingolipid and sulfatide molecular species in progressive epilepsy with mental retardation, EPMR, brain: a case study. J Neurochem. 2005;95:609-17 pubmed
  27. Bodennec J, Trajkovic Bodennec S, Futerman A. Simultaneous quantification of lyso-neutral glycosphingolipids and neutral glycosphingolipids by N-acetylation with [3H]acetic anhydride. J Lipid Res. 2003;44:1413-9 pubmed
    We describe a new method that permits quantification in the pmol to nmol range of three lyso-neutral glycosphingolipids (lyso-n-GSLs), glucosylsphingosine (GlcSph), galactosylsphingosine (GalSph), and lactosylsphingosine, in the same ..
  28. Vastagh I, Constantin T, Kéri A, Rudas G, Fekete G, Bereczki D. [Neurological complications of Fabry-disease]. Ideggyogy Sz. 2011;64:29-35 pubmed
    ..Deficiency of alpha-galactosidase A activity leads to the accumulation of neutral glycosphingolipids, primarily globotriaosylceramide (GL-3) in various tissues, particularly blood vessels, kidneys, ..
  29. Vukelic Z, Kalanj Bognar S. Cell density-dependent changes of glycosphingolipid biosynthesis in cultured human skin fibroblasts. Glycoconj J. 2001;18:429-37 pubmed
    ..100%) of sparse cell populations showed a significant predominance of the gangliosides (70%) over the neutral glycosphingolipids (30%), with ganglioside GM2 as the major species followed by monohexosyl-ceramide...
  30. De Rosa M, Sillence D, Ackerley C, Lingwood C. Role of multiple drug resistance protein 1 in neutral but not acidic glycosphingolipid biosynthesis. J Biol Chem. 2004;279:7867-76 pubmed
    ..These studies identify MDR1 as the major glucosyl ceramide flippase required for neutral glycosphingolipid anabolism and demonstrate a previously unappreciated dichotomy between neutral and acid glycosphingolipid synthesis. ..
  31. Markotic A, Marusic A, Muthing J. Expression of neutral glycosphingolipids in the brain, lymphoid organs and lungs of mice lacking beta(2)-microglobulin. Neuroimmunomodulation. 2005;12:310-3 pubmed
  32. Lee Y, Lee B, Jeon H, Kim K. Total synthesis of agelagalastatin. Org Lett. 2006;8:3971-4 pubmed
    ..The trisaccharide component was constructed employing the CB glycoside method which permitted a completely alpha-stereoselective galactofuranosylation...
  33. Abe A, Arend L, Lee L, Lingwood C, Brady R, Shayman J. Glycosphingolipid depletion in fabry disease lymphoblasts with potent inhibitors of glucosylceramide synthase. Kidney Int. 2000;57:446-54 pubmed
    ..We suggest that these compounds have potential therapeutic utility in the treatment of Fabry disease. ..
  34. Meivar Levy I, Futerman A. Up-regulation of neutral glycosphingolipid synthesis upon long term inhibition of ceramide synthesis by fumonisin B1. J Biol Chem. 1999;274:4607-12 pubmed
    ..These data demonstrate that GSL synthesis and in particular Gb3 synthesis are tightly regulated in fibroblasts, presumably so as to maintain constant levels of Gb3 on the cell surface. ..
  35. Abeytunga D, Oland L, Somogyi A, Polt R. Structural studies on the neutral glycosphingolipids of Manduca sexta. Bioorg Chem. 2008;36:70-6 pubmed
    ..The ceramide trisaccharides and ceramide tetrasaccharide belong to the arthro-series, The observation of fucose in the M. sexta GSLs is the first report of the presence of fucose in an arthroseries GSL. ..