Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-02-09 00:07:46 UTC
HMDB IDHMDB00024
Secondary Accession NumbersNone
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
Thumb
Synonyms
  1. 3'-O-Sulphogalactosylceramide
  2. 3-O-Sulfo-beta-D-galactosylceramide
  3. 3-O-Sulfo-beta-delta-galactosylceramide
  4. 3-O-Sulfogalactosylceramide
  5. 3-O-Sulphogalactosylceramide
  6. Cerebroside 3-sulfate
  7. Cerebroside 3-sulphate
  8. Galactosylceramide-sulfate
  9. Galactosylceramide-sulphate
  10. Galactosylceramidesulfate
  11. Galactosylceramidesulphate
  12. N-[(1S,2R,3E)-2-Hydroxy-1-[[(3-O-sulfo-b-D-galactopyranosyl)oxy]methyl]-3-heptadecen-1-yl]-Tetracosanamide
  13. N-[(1S,2R,3E)-2-Hydroxy-1-[[(3-O-sulfo-b-D-galactopyranosyl)oxy]methyl]-3-heptadecenyl]-Tetracosanamide
  14. N-[(1S,2R,3E)-2-Hydroxy-1-[[(3-O-sulfo-beta-delta-galactopyranosyl)oxy]methyl]-3-heptadecen-1-yl]-Tetracosanamide
  15. N-[(1S,2R,3E)-2-Hydroxy-1-[[(3-O-sulfo-beta-delta-galactopyranosyl)oxy]methyl]-3-heptadecenyl]-Tetracosanamide
  16. Sulfatide
  17. Sulfatide (d18:1/24:0)
  18. [R-[R*,S*-(E)]]-N-[2-hydroxy-1-[[(3-O-sulfo-b-D-galactopyranosyl)oxy]methyl]-3-heptadecenyl]-Tetracosanamide
  19. [R-[R*,S*-(E)]]-N-[2-hydroxy-1-[[(3-O-sulfo-beta-delta-galactopyranosyl)oxy]methyl]-3-heptadecenyl]-Tetracosanamide
Chemical FormulaC48H93NO11S
Average Molecular Weight892.317
Monoisotopic Molecular Weight891.646933513
IUPAC Name[(2R,3R,4S,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
Traditional IUPAC Name[(2R,3R,4S,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
CAS Registry Number151122-71-3
SMILES
CCCCCCCCCCCCCCCCCCCCCCCC(=O)N[C@@H](CO[C@@H]1O[C@H](CO)[C@H](O)[C@H](OS(O)(=O)=O)[C@H]1O)[C@H](O)\C=C\CCCCCCCCCCCCC
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
KingdomOrganic Compounds
Super ClassLipids
ClassSphingolipids
Sub ClassSulfatides
Other Descriptors
  • Aliphatic Heteromonocyclic Compounds
Substituents
  • Acetal
  • Alkyl Glycoside
  • Allyl Alcohol
  • Carboxamide Group
  • Ceramide
  • Fatty Acyl Glycoside
  • Glycosyl Compound
  • Hexose Monosaccharide
  • N Acyl Amine
  • O Glycosyl Compound
  • Organic Sulfuric Acid Monoester
  • Oxane
  • Primary Alcohol
  • Saccharide
  • Secondary Alcohol
  • Secondary Carboxylic Acid Amide
  • Sulfate Ester
Direct ParentSulfatides
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Component of Glycosphingolipid metabolism
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
  • Lysosome
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
water solubility2.090E-04 g/LALOGPS
logP6.01ALOGPS
logP10.65ChemAxon
logS-6.6ALOGPS
pKa (strongest acidic)-1.9ChemAxon
pKa (strongest basic)0.025ChemAxon
physiological charge-1ChemAxon
hydrogen acceptor count10ChemAxon
hydrogen donor count6ChemAxon
polar surface area192.08ChemAxon
rotatable bond count43ChemAxon
refractivity244.99ChemAxon
polarizability111.22ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Lysosome
Biofluid Locations
  • Urine
Tissue Location
  • Brain
  • Kidney
  • Myelin
Pathways
NameSMPDB LinkKEGG Link
Sphingolipid MetabolismSMP00034map00500
Normal Concentrations
BiofluidStatusValueAgeSexConditionReference
UrineDetected and Quantified0.205 (0.07-0.34) umol/mmol creatinineAdult (>18 years old)BothNormal
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReference
UrineDetected and Quantified12.7 (1.2-24.2) umol/mmol creatinineAdult (>18 years old)BothMetachromatic Leukodystrophy (MLD)
Associated Disorders and Diseases
Disease References
Metachromatic leukodystrophy
  • 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)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021878
KNApSAcK IDNot Available
Chemspider ID4953605
KEGG Compound IDC06125
BioCyc IDGALACTOSYLCERAMIDE-SULFATE
BiGG IDNot Available
Wikipedia LinkSulfatide
NuGOwiki LinkHMDB00024
Metagene LinkHMDB00024
METLIN ID5096
PubChem Compound6451121
PDB IDNot Available
ChEBI ID60361
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] Nippon 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

Only showing the first 50 proteins. There are 71 proteins in total.

Enzymes

Gene Name:
GALC
Uniprot ID:
P54803
Gene Name:
ARSD
Uniprot ID:
P51689
Gene Name:
GAL3ST1
Uniprot ID:
Q99999
Gene Name:
ARSA
Uniprot ID:
P15289
Gene Name:
ARSE
Uniprot ID:
P51690
Gene Name:
SMPD1
Uniprot ID:
P17405
Gene Name:
UGCG
Uniprot ID:
Q16739
Gene Name:
ASAH1
Uniprot ID:
Q13510
Gene Name:
SMPD2
Uniprot ID:
O60906
Gene Name:
COL4A3BP
Uniprot ID:
Q9Y5P4
Gene Name:
PSAP
Uniprot ID:
P07602
Gene Name:
GM2A
Uniprot ID:
P17900
Gene Name:
MBP
Uniprot ID:
P02686
Gene Name:
GBA
Uniprot ID:
P04062
Gene Name:
SELP
Uniprot ID:
P16109
Gene Name:
SELL
Uniprot ID:
P14151
Gene Name:
NPC2
Uniprot ID:
P61916
Gene Name:
CERS1
Uniprot ID:
P27544
Gene Name:
CD1D
Uniprot ID:
P15813
Gene Name:
ADAMTSL1
Uniprot ID:
Q8N6G6
Gene Name:
PIGM
Uniprot ID:
Q9H3S5
Gene Name:
PIGZ
Uniprot ID:
Q86VD9
Gene Name:
SMPD3
Uniprot ID:
Q9NY59
Gene Name:
B3GALT5
Uniprot ID:
Q9Y2C3
Gene Name:
B4GALT6
Uniprot ID:
Q9UBX8
Gene Name:
PIGB
Uniprot ID:
Q92521
Gene Name:
PIGS
Uniprot ID:
Q96S52
Gene Name:
PIGT
Uniprot ID:
Q969N2
Gene Name:
PIGV
Uniprot ID:
Q9NUD9
Gene Name:
SMPD4
Uniprot ID:
Q9NXE4

Only showing the first 50 proteins. There are 71 proteins in total.