The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
Chemical Compound Review

Galactosylsphingosine     (2R,3R,4S,5S,6R)-2-[(2S,3R)- 2-amino-3...

Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Galactosylsphingosine


High impact information on Galactosylsphingosine

  • Concomitantly, the levels of psychosine, a highly toxic lipid that progressively accumulates in the CNS of untreated twitcher mice, stabilized at much lower levels in the CNS of treated twitcher mice [6].
  • Treatment of cultured cells expressing this receptor with psychosine or structurally related glycosphingolipids results in the formation of globoid, multinuclear cells [7].
  • Our discovery of a molecular target for psychosine suggests a mechanism for the globoid cell histology characteristic of GLD, provides a tool with which to explore the disjunction of mitosis and cytokinesis in cell cultures, and provides a platform for developing a medicinal chemistry for psychosine [7].
  • When psychosine was added to the human myelomonocyte cell line U937, which was the most sensitive among the cell lines tested, cleavage furrow formed either incompletely or almost completely [8].
  • Psychosine treatment also induced giant clots of actin filaments in the cells that probably consisted of small vacuoles with filamentous structures, suggesting that psychosine affected actin reorganization [8].

Chemical compound and disease context of Galactosylsphingosine


Biological context of Galactosylsphingosine

  • Inhibition of cytokinesis by a lipid metabolite, psychosine [8].
  • On the other hand, brain psychosine level is increased in all twitcher mice, but galc(-/-), bgal(+/-) mice show a significantly higher level than other genotypes [13].
  • Activation of TDAG8 by its agonist psychosine markedly enhanced dexamethasone-induced apoptosis in a TDAG8-dependent manner [14].
  • Although IGF-1 sustained phosphorylation of both of these pathways, it provided maximum protection to OLP-II cells from psychosine-induced cell death in a PI3K/Akt-dependent manner [15].
  • Methods for isolation of neutral lysoglycosphingolipids (n-lyso-GSLs) such as glucosylsphingosine and galactosylsphingosine normally involve mild alkaline or acid hydrolysis followed by multiple chromatography steps, yielding relatively low recoveries of n-lyso-GSLs and neutral glycosphingolipids (n-GSLs) [16].

Anatomical context of Galactosylsphingosine


Associations of Galactosylsphingosine with other chemical compounds


Gene context of Galactosylsphingosine


Analytical, diagnostic and therapeutic context of Galactosylsphingosine


  1. The twitcher mouse: a model for Krabbe disease and for experimental therapies. Suzuki, K., Suzuki, K. Brain Pathol. (1995) [Pubmed]
  2. Pertussis toxin inhibits phospholipase C activation and Ca2+ mobilization by sphingosylphosphorylcholine and galactosylsphingosine in HL60 leukemia cells. Implications of GTP-binding protein-coupled receptors for lysosphingolipids. Okajima, F., Kondo, Y. J. Biol. Chem. (1995) [Pubmed]
  3. Galactosylceramidase deficiency causes sperm abnormalities in the mouse model of globoid cell leukodystrophy. Luddi, A., Strazza, M., Carbone, M., Moretti, E., Costantino-Ceccarini, E. Exp. Cell Res. (2005) [Pubmed]
  4. Molecular mechanism of psychosine-induced cell death in human oligodendrocyte cell line. Haq, E., Giri, S., Singh, I., Singh, A.K. J. Neurochem. (2003) [Pubmed]
  5. Accumulation of glucosylceramide and glucosylsphingosine (psychosine) in cerebrum and cerebellum in infantile and juvenile Gaucher disease. Nilsson, O., Svennerholm, L. J. Neurochem. (1982) [Pubmed]
  6. Donor-derived cells in the central nervous system of twitcher mice after bone marrow transplantation. Hoogerbrugge, P.M., Suzuki, K., Suzuki, K., Poorthuis, B.J., Kobayashi, T., Wagemaker, G., van Bekkum, D.W. Science (1988) [Pubmed]
  7. Identification of a molecular target of psychosine and its role in globoid cell formation. Im, D.S., Heise, C.E., Nguyen, T., O'Dowd, B.F., Lynch, K.R. J. Cell Biol. (2001) [Pubmed]
  8. Inhibition of cytokinesis by a lipid metabolite, psychosine. Kanazawa, T., Nakamura, S., Momoi, M., Yamaji, T., Takematsu, H., Yano, H., Sabe, H., Yamamoto, A., Kawasaki, T., Kozutsumi, Y. J. Cell Biol. (2000) [Pubmed]
  9. Krabbe disease: psychosine-mediated activation of phospholipase A2 in oligodendrocyte cell death. Giri, S., Khan, M., Rattan, R., Singh, I., Singh, A.K. J. Lipid Res. (2006) [Pubmed]
  10. Lysosulfatide (sulfogalactosylsphingosine) accumulation in tissues from patients with metachromatic leukodystrophy. Toda, K., Kobayashi, T., Goto, I., Ohno, K., Eto, Y., Inui, K., Okada, S. J. Neurochem. (1990) [Pubmed]
  11. Purification and characterization of 3'-phosphoadenosine-5'-phosphosulfate:GalCer sulfotransferase from human renal cancer cells. Honke, K., Yamane, M., Ishii, A., Kobayashi, T., Makita, A. J. Biochem. (1996) [Pubmed]
  12. Biochemical pathogenesis of demyelination in globoid cell leukodystrophy (Krabbe's disease): the effects of psychosine upon oligodendroglial cell culture. Ida, H., Eto, Y., Maekawa, K. Acta paediatrica Japonica; Overseas edition. (1990) [Pubmed]
  13. Paradoxical influence of acid beta-galactosidase gene dosage on phenotype of the twitcher mouse (genetic galactosylceramidase deficiency). Tohyama, J., Vanier, M.T., Suzuki, K., Ezoe, T., Matsuda, J., Suzuki, K. Hum. Mol. Genet. (2000) [Pubmed]
  14. The glucocorticoid-induced gene tdag8 encodes a pro-apoptotic G protein-coupled receptor whose activation promotes glucocorticoid-induced apoptosis. Malone, M.H., Wang, Z., Distelhorst, C.W. J. Biol. Chem. (2004) [Pubmed]
  15. Insulin-like growth factor-1 provides protection against psychosine-induced apoptosis in cultured mouse oligodendrocyte progenitor cells using primarily the PI3K/Akt pathway. Zaka, M., Rafi, M.A., Rao, H.Z., Luzi, P., Wenger, D.A. Mol. Cell. Neurosci. (2005) [Pubmed]
  16. Aminopropyl solid phase extraction and 2 D TLC of neutral glycosphingolipids and neutral lysoglycosphingolipids. Bodennec, J., Pelled, D., Futerman, A.H. J. Lipid Res. (2003) [Pubmed]
  17. Hematopoietic cell transplantation in murine globoid cell leukodystrophy (the twitcher mouse): effects on levels of galactosylceramidase, psychosine, and galactocerebrosides. Ichioka, T., Kishimoto, Y., Brennan, S., Santos, G.W., Yeager, A.M. Proc. Natl. Acad. Sci. U.S.A. (1987) [Pubmed]
  18. Normal immune development and glucocorticoid-induced thymocyte apoptosis in mice deficient for the T-cell death-associated gene 8 receptor. Radu, C.G., Cheng, D., Nijagal, A., Riedinger, M., McLaughlin, J., Yang, L.V., Johnson, J., Witte, O.N. Mol. Cell. Biol. (2006) [Pubmed]
  19. Infantile and fetal globoid cell leukodystrophy: analysis of galactosylceramide and galactosylsphingosine. Kobayashi, T., Goto, I., Yamanaka, T., Suzuki, Y., Nakano, T., Suzuki, K. Ann. Neurol. (1988) [Pubmed]
  20. Murine, canine and non-human primate models of Krabbe disease. Wenger, D.A. Molecular medicine today. (2000) [Pubmed]
  21. TDAG8 is a proton-sensing and psychosine-sensitive G-protein-coupled receptor. Wang, J.Q., Kon, J., Mogi, C., Tobo, M., Damirin, A., Sato, K., Komachi, M., Malchinkhuu, E., Murata, N., Kimura, T., Kuwabara, A., Wakamatsu, K., Koizumi, H., Uede, T., Tsujimoto, G., Kurose, H., Sato, T., Harada, A., Misawa, N., Tomura, H., Okajima, F. J. Biol. Chem. (2004) [Pubmed]
  22. Glycosphingolipid-induced relocation of Lyn and Syk into detergent-resistant membranes results in mast cell activation. Prieschl, E.E., Csonga, R., Novotny, V., Kikuchi, G.E., Baumruker, T. J. Immunol. (2000) [Pubmed]
  23. Chromatographic resolution and quantitative assay of CNS tissue sphingoids and sphingolipids. Dasgupta, S., Hogan, E.L. J. Lipid Res. (2001) [Pubmed]
  24. Krabbe disease: genetic aspects and progress toward therapy. Wenger, D.A., Rafi, M.A., Luzi, P., Datto, J., Costantino-Ceccarini, E. Mol. Genet. Metab. (2000) [Pubmed]
  25. Decreased fatty acylation of myelin proteolipid protein in the twitcher mouse. Yoshimura, T., Kobayashi, T., Mitsuo, K., Goto, I. J. Neurochem. (1989) [Pubmed]
  26. Novel procedure for measuring psychosine derivatives by an HPLC method. Nozawa, M., Iwamoto, T., Tokoro, T., Eto, Y. J. Neurochem. (1992) [Pubmed]
  27. Effect of chain unsaturation on the structure and thermotropic properties of galactocerebrosides. Reed, R.A., Shipley, G.G. Biophys. J. (1989) [Pubmed]
  28. Physicochemical characterization of psychosine by 1H nuclear magnetic resonance and electron microscopy. Orfi, L., Larive, C.K., LeVine, S.M. Lipids (1997) [Pubmed]
  29. Binding of galactosylsphingosine (psychosine) by albumin. Igisu, H., Matsuoka, M., Hamasaki, N. Lipids (1990) [Pubmed]
WikiGenes - Universities