Record Information |
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Version | 5.0 |
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Status | Detected and Quantified |
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Creation Date | 2005-11-16 15:48:42 UTC |
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Update Date | 2022-03-07 02:48:59 UTC |
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HMDB ID | HMDB0000024 |
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Secondary Accession Numbers | |
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Metabolite Identification |
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Common Name | 3-O-Sulfogalactosylceramide (d18:1/24:0) |
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Description | 3-O-Sulfogalactosylceramide is an acidic, sulfated glycosphingolipid, often known as sulfatide. This lipid occurs in membranes of various cell types, but is found in particularly high concentrations in myelin where it constitutes 3-4% of total membrane lipids. This lipid is synthesized primarily in the oligodendrocytes in the central nervous system. Accumulation of this lipid in the lysosomes is a characteristic of metachromatic leukodystrophy, a lysosomal storage disease caused by the deficiency of arylsulfatase A. Alterations in sulfatide metabolism, trafficking, and homeostasis are present in the earliest clinically recognizable stages of Alzheimer's disease. Cerebrosides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Cerebrosides have a single sugar group linked to ceramide. The most common are galactocerebrosides (containing galactose), the least common are glucocerebrosides (containing glucose). Galactocerebrosides are found predominantly in neuronal cell membranes. In contrast glucocerebrosides are not normally found in membranes. Instead, they are typically intermediates in the synthesis or degradation of more complex glycosphingolipids. Galactocerebrosides are synthesized from ceramide and UDP-galactose. Excess lysosomal accumulation of glucocerebrosides is found in Gaucher disease. Sulfatides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Sulfatides are the sulfuric acid esters of galactocerebrosides. They are synthesized from galactocerebrosides and activated sulfate, 3'-phosphoadenosine 5'-phosphosulfate (PAPS). |
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Structure | [H][C@@](CO[C@@H]1O[C@H](CO)[C@H](O)C(OS(O)(=O)=O)C1O)(NC(=O)CCCCCCCCCCCCCCCCCCCCCCC)[C@]([H])(O)\C=C\CCCCCCCCCCCCC InChI=1S/C48H93NO11S/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-23-24-26-28-30-32-34-36-38-44(52)49-41(42(51)37-35-33-31-29-27-25-16-14-12-10-8-6-4-2)40-58-48-46(54)47(60-61(55,56)57)45(53)43(39-50)59-48/h35,37,41-43,45-48,50-51,53-54H,3-34,36,38-40H2,1-2H3,(H,49,52)(H,55,56,57)/b37-35+/t41-,42+,43+,45-,46?,47?,48+/m0/s1 |
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Synonyms | Value | Source |
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3-O-Sulphogalactosylceramide (D18:1/24:0) | Generator |
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Chemical Formula | C48H93NO11S |
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Average Molecular Weight | 892.33 |
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Monoisotopic Molecular Weight | 891.646933993 |
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IUPAC Name | [(2R,5S,6R)-3,5-dihydroxy-2-{[(2S,3R,4E)-3-hydroxy-2-tetracosanamidooctadec-4-en-1-yl]oxy}-6-(hydroxymethyl)oxan-4-yl]oxidanesulfonic acid |
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Traditional Name | C24 sulfatide |
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CAS Registry Number | 151122-71-3 |
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SMILES | [H][C@@](CO[C@@H]1O[C@H](CO)[C@H](O)C(OS(O)(=O)=O)C1O)(NC(=O)CCCCCCCCCCCCCCCCCCCCCCC)[C@]([H])(O)\C=C\CCCCCCCCCCCCC |
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InChI Identifier | InChI=1S/C48H93NO11S/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-23-24-26-28-30-32-34-36-38-44(52)49-41(42(51)37-35-33-31-29-27-25-16-14-12-10-8-6-4-2)40-58-48-46(54)47(60-61(55,56)57)45(53)43(39-50)59-48/h35,37,41-43,45-48,50-51,53-54H,3-34,36,38-40H2,1-2H3,(H,49,52)(H,55,56,57)/b37-35+/t41-,42+,43+,45-,46?,47?,48+/m0/s1 |
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InChI Key | MEAZTWJVOWHKJM-YQTIZROBSA-N |
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Chemical Taxonomy |
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Description | Belongs to the class of organic compounds known as methoxyphenols. Methoxyphenols are compounds containing a methoxy group attached to the benzene ring of a phenol moiety. |
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Kingdom | Organic compounds |
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Super Class | Benzenoids |
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Class | Phenols |
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Sub Class | Methoxyphenols |
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Direct Parent | Methoxyphenols |
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Alternative Parents | |
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Substituents | - Methoxyphenol
- Phenethylamine
- Anisole
- Phenoxy compound
- Phenol ether
- Methoxybenzene
- 2-arylethylamine
- Aralkylamine
- Alkyl aryl ether
- 1-hydroxy-2-unsubstituted benzenoid
- Monocyclic benzene moiety
- Ether
- Primary amine
- Amine
- Organooxygen compound
- Organonitrogen compound
- Primary aliphatic amine
- Organic nitrogen compound
- Hydrocarbon derivative
- Organopnictogen compound
- Organic oxygen compound
- Aromatic homomonocyclic compound
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Molecular Framework | Aromatic homomonocyclic compounds |
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External Descriptors | |
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Ontology |
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Not Available | Not Available |
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Physical Properties |
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State | Solid |
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Experimental Molecular Properties | Property | Value | Reference |
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Melting Point | Not Available | Not Available | Boiling Point | Not Available | Not Available | Water Solubility | Not Available | Not Available | LogP | Not Available | Not Available |
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Experimental Chromatographic Properties | Not Available |
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Predicted Molecular Properties | |
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Predicted Chromatographic Properties | Predicted Collision Cross SectionsPredicted Kovats Retention IndicesUnderivatized |
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Spectra |
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| MS/MS SpectraSpectrum Type | Description | Splash Key | Deposition Date | Source | View |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 3-O-Sulfogalactosylceramide (d18:1/24:0) 10V, Negative-QTOF | splash10-0006-0010000090-4009dca32137df81dfe1 | 2021-09-23 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 3-O-Sulfogalactosylceramide (d18:1/24:0) 20V, Negative-QTOF | splash10-000x-2093004070-440431caa30e54f5b02f | 2021-09-23 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 3-O-Sulfogalactosylceramide (d18:1/24:0) 40V, Negative-QTOF | splash10-052b-9115315000-b669e31b7b81f310731f | 2021-09-23 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 3-O-Sulfogalactosylceramide (d18:1/24:0) 10V, Positive-QTOF | splash10-000x-3001009080-1e911bc6ce858fa13092 | 2021-09-25 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 3-O-Sulfogalactosylceramide (d18:1/24:0) 20V, Positive-QTOF | splash10-001i-4011009010-ee22cc729d52a1645af6 | 2021-09-25 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 3-O-Sulfogalactosylceramide (d18:1/24:0) 40V, Positive-QTOF | splash10-06dl-7951023000-4afdf97295dee65586e4 | 2021-09-25 | Wishart Lab | View Spectrum |
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Biological Properties |
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Cellular Locations | - Extracellular
- Membrane
- Lysosome
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Biospecimen Locations | |
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Tissue Locations | |
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Pathways | |
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Normal Concentrations |
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Urine | Detected and Quantified | 0.205 (0.07-0.34) umol/mmol creatinine | Adult (>18 years old) | Both | Normal | | details |
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Abnormal Concentrations |
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Urine | Detected and Quantified | 12.7 (1.2-24.2) umol/mmol creatinine | Adult (>18 years old) | Both | Metachromatic Leukodystrophy (MLD) | | details |
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Associated Disorders and Diseases |
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Disease References | Metachromatic leukodystrophy |
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- Natowicz MR, Prence EM, Chaturvedi P, Newburg DS: Urine sulfatides and the diagnosis of metachromatic leukodystrophy. Clin Chem. 1996 Feb;42(2):232-8. [PubMed:8595716 ]
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Associated OMIM IDs | - 250100 (Metachromatic leukodystrophy)
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External Links |
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DrugBank ID | Not Available |
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Phenol Explorer Compound ID | Not Available |
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FooDB ID | Not Available |
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KNApSAcK ID | Not Available |
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Chemspider ID | 24823255 |
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KEGG Compound ID | Not Available |
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BioCyc ID | Not Available |
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BiGG ID | Not Available |
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Wikipedia Link | Not Available |
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METLIN ID | Not Available |
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PubChem Compound | 24779585 |
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PDB ID | Not Available |
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ChEBI ID | Not Available |
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Food Biomarker Ontology | Not Available |
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VMH ID | Not Available |
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MarkerDB ID | MDB00000013 |
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Good Scents ID | Not Available |
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References |
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Synthesis Reference | Hakomori, Senichiro; Ishimoda, Taiko; Nakamura, Kiyoshi. Separation and characterization of two sulfatides of brain. Journal of Biochemistry (Tokyo, Japan) (1962), 52 468-9. |
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Material Safety Data Sheet (MSDS) | Not Available |
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General References | - Wenger DA, DeGala G, Williams C, Taylor HA, Stevenson RE, Pruitt JR, Miller J, Garen PD, Balentine JD: Clinical, pathological, and biochemical studies on an infantile case of sulfatide/GM1 activator protein deficiency. Am J Med Genet. 1989 Jun;33(2):255-65. [PubMed:2764035 ]
- Tamaoki A, Kikkawa Y: [The role of sulfatides in autoimmunity in children with various glomerular disease]. Nihon Jinzo Gakkai Shi. 1991 Nov;33(11):1045-54. [PubMed:1808356 ]
- Eto Y, Kawame H, Hasegawa Y, Ohashi T, Ida H, Tokoro T: Molecular characteristics in Japanese patients with lipidosis: novel mutations in metachromatic leukodystrophy and Gaucher disease. Mol Cell Biochem. 1993 Feb 17;119(1-2):179-84. [PubMed:8455580 ]
- Monti E, Preti A, Novati A, Aleo MF, Clemente ML, Marchesini S: Uptake and metabolism of a fluorescent sulfatide analogue in cultured skin fibroblasts. Biochim Biophys Acta. 1992 Feb 20;1124(1):80-7. [PubMed:1543730 ]
- Tsaioun KI: Vitamin K-dependent proteins in the developing and aging nervous system. Nutr Rev. 1999 Aug;57(8):231-40. [PubMed:10518409 ]
- Nobile-Orazio E, Manfredini E, Carpo M, Meucci N, Monaco S, Ferrari S, Bonetti B, Cavaletti G, Gemignani F, Durelli L, et al.: Frequency and clinical correlates of anti-neural IgM antibodies in neuropathy associated with IgM monoclonal gammopathy. Ann Neurol. 1994 Sep;36(3):416-24. [PubMed:8080249 ]
- Natowicz MR, Prence EM, Chaturvedi P, Newburg DS: Urine sulfatides and the diagnosis of metachromatic leukodystrophy. Clin Chem. 1996 Feb;42(2):232-8. [PubMed:8595716 ]
- Hulyalkar AR, Nora R, Manowitz P: Arylsulfatase A variants in patients with alcoholism. Alcohol Clin Exp Res. 1984 May-Jun;8(3):337-41. [PubMed:6146271 ]
- Marchesini S, Preti A, Aleo MF, Casella A, Dagan A, Gatt S: Synthesis, spectral properties and enzymatic hydrolysis of fluorescent derivatives of cerebroside sulfate containing long-wavelength-emission probes. Chem Phys Lipids. 1990 Mar;53(2-3):165-75. [PubMed:1970953 ]
- Cheng H, Xu J, McKeel DW Jr, Han X: Specificity and potential mechanism of sulfatide deficiency in Alzheimer's disease: an electrospray ionization mass spectrometric study. Cell Mol Biol (Noisy-le-grand). 2003 Jul;49(5):809-18. [PubMed:14528918 ]
- Guchhait P, Lopez JA, Thiagarajan P: Characterization of autoantibodies against sulfatide from a V-gene phage-display library derived from patients with systemic lupus erythematosus. J Immunol Methods. 2004 Dec;295(1-2):129-37. Epub 2004 Oct 26. [PubMed:15627618 ]
- Eto Y, Tahara T, Koda N, Yamaguchi S, Ito F, Okuno A: Prenatal diagnosis of metachromatic leukodystrophy: a diagnosis by amniotic fluid and its confirmation. Arch Neurol. 1982 Jan;39(1):29-32. [PubMed:6119972 ]
- Inui K, Wenger DA: Concentrations of an activator protein for sphingolipid hydrolysis in liver and brain samples from patients with lysosomal storage diseases. J Clin Invest. 1983 Nov;72(5):1622-8. [PubMed:6415115 ]
- Li YT, Muhiudeen IA, DeGasperi R, Hirabayashi Y, Li SC: Presence of activator proteins for the enzymic hydrolysis of GM1 and GM2 gangliosides in normal human urine. Am J Hum Genet. 1983 Jul;35(4):629-34. [PubMed:6881139 ]
- Matzner U, Herbst E, Hedayati KK, Lullmann-Rauch R, Wessig C, Schroder S, Eistrup C, Moller C, Fogh J, Gieselmann V: Enzyme replacement improves nervous system pathology and function in a mouse model for metachromatic leukodystrophy. Hum Mol Genet. 2005 May 1;14(9):1139-52. Epub 2005 Mar 16. [PubMed:15772092 ]
- Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
- Watson AD: Thematic review series: systems biology approaches to metabolic and cardiovascular disorders. Lipidomics: a global approach to lipid analysis in biological systems. J Lipid Res. 2006 Oct;47(10):2101-11. Epub 2006 Aug 10. [PubMed:16902246 ]
- Sethi JK, Vidal-Puig AJ: Thematic review series: adipocyte biology. Adipose tissue function and plasticity orchestrate nutritional adaptation. J Lipid Res. 2007 Jun;48(6):1253-62. Epub 2007 Mar 20. [PubMed:17374880 ]
- Lingwood D, Simons K: Lipid rafts as a membrane-organizing principle. Science. 2010 Jan 1;327(5961):46-50. doi: 10.1126/science.1174621. [PubMed:20044567 ]
- Divecha N, Irvine RF: Phospholipid signaling. Cell. 1995 Jan 27;80(2):269-78. [PubMed:7834746 ]
- Ghosh S, Strum JC, Bell RM: Lipid biochemistry: functions of glycerolipids and sphingolipids in cellular signaling. FASEB J. 1997 Jan;11(1):45-50. [PubMed:9034165 ]
- Cevc, Gregor (1993). Phospholipids Handbook. Marcel Dekker.
- Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.
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