You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-01-11 19:13:58 UTC
HMDB IDHMDB0000024
Secondary Accession Numbers
  • HMDB00024
Metabolite Identification
Common Name3-O-Sulfogalactosylceramide (d18:1/24:0)
Description3-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).
Structure
Data?1547234038
SynonymsNot Available
Chemical FormulaC48H93NO11S
Average Molecular Weight892.317
Monoisotopic Molecular Weight891.646933513
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry Number151122-71-3
SMILESNot Available
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
InChI KeyMEAZTWJVOWHKJM-CIAPRIGGSA-N
Chemical Taxonomy
ClassificationNot classified
Ontology
Physiological effect

Organoleptic effect:

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted PropertiesNot Available
Spectra
Spectrum TypeDescriptionSplash KeyView
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Lysosome
Biospecimen Locations
  • Urine
Tissue Locations
  • Brain
  • Kidney
  • Myelin
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified0.205 (0.07-0.34) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified12.7 (1.2-24.2) umol/mmol creatinineAdult (>18 years old)BothMetachromatic Leukodystrophy (MLD) details
Associated Disorders and Diseases
Disease References
Metachromatic leukodystrophy
  1. 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 ]
Associated OMIM IDs
  • 250100 (Metachromatic leukodystrophy)
External LinksNot Available
References
Synthesis ReferenceHakomori, Senichiro; Ishimoda, Taiko; Nakamura, Kiyoshi. Separation and characterization of two sulfatides of brain. Journal of Biochemistry (Tokyo, Japan) (1962), 52 468-9.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. Tsaioun KI: Vitamin K-dependent proteins in the developing and aging nervous system. Nutr Rev. 1999 Aug;57(8):231-40. [PubMed:10518409 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. 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 ]
  11. 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 ]
  12. 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 ]
  13. 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 ]
  14. 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 ]
  15. 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 ]
  16. Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
  17. 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 ]
  18. 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 ]
  19. 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 ]
  20. Divecha N, Irvine RF: Phospholipid signaling. Cell. 1995 Jan 27;80(2):269-78. [PubMed:7834746 ]
  21. 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 ]
  22. Cevc, Gregor (1993). Phospholipids Handbook. Marcel Dekker.
  23. Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.

Only showing the first 10 proteins. There are 71 proteins in total.

Enzymes

General function:
Involved in galactosylceramidase activity
Specific function:
Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.
Gene Name:
GALC
Uniprot ID:
P54803
Molecular weight:
77062.86
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
ARSD
Uniprot ID:
P51689
Molecular weight:
64859.3
General function:
Involved in galactosylceramide sulfotransferase activity
Specific function:
Catalyzes the sulfation of membrane glycolipids. Seems to prefer beta-glycosides at the non-reducing termini of sugar chains attached to a lipid moiety. Catalyzes the synthesis of galactosylceramide sulfate (sulfatide), a major lipid component of the myelin sheath and of monogalactosylalkylacylglycerol sulfate (seminolipid), present in spermatocytes (By similarity). Also acts on lactosylceramide, galactosyl 1-alkyl-2-sn-glycerol and galactosyl diacylglycerol (in vitro).
Gene Name:
GAL3ST1
Uniprot ID:
Q99999
Molecular weight:
48763.63
General function:
Involved in catalytic activity
Specific function:
Hydrolyzes cerebroside sulfate.
Gene Name:
ARSA
Uniprot ID:
P15289
Molecular weight:
53805.87
General function:
Involved in catalytic activity
Specific function:
May be essential for the correct composition of cartilage and bone matrix during development. Has no activity toward steroid sulfates
Gene Name:
ARSE
Uniprot ID:
P51690
Molecular weight:
65668.4
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
Catalyzes the formation of some glycolipid via the addition of N-acetylgalactosamine (GalNAc) in alpha-1,3-linkage to some substrate. Glycolipids probably serve for adherence of some pathogens
Gene Name:
GBGT1
Uniprot ID:
Q8N5D6
Molecular weight:
40126.9
General function:
Involved in N-acetylglucosaminylphosphatidylinositol de
Specific function:
Involved in the second step of GPI biosynthesis. De-N-acetylation of N-acetylglucosaminyl-phosphatidylinositol.
Gene Name:
PIGL
Uniprot ID:
Q9Y2B2
Molecular weight:
28530.965
General function:
Involved in hydrolase activity
Specific function:
Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.
Gene Name:
SMPD1
Uniprot ID:
P17405
Molecular weight:
69935.53
General function:
Cell wall/membrane/envelope biogenesis
Specific function:
Catalyzes the first glycosylation step in glycosphingolipid biosynthesis, the transfer of glucose to ceramide. May also serve as a "flippase".
Gene Name:
UGCG
Uniprot ID:
Q16739
Molecular weight:
44853.255
General function:
Involved in galactosyltransferase activity
Specific function:
Necessary for the biosynthesis of the Pk antigen of blood histogroup P. Catalyzes the transfer of galactose to lactosylceramide and galactosylceramide. Necessary for the synthesis of the receptor for bacterial verotoxins.
Gene Name:
A4GALT
Uniprot ID:
Q9NPC4
Molecular weight:
40498.78

Only showing the first 10 proteins. There are 71 proteins in total.