| Record Information |
|---|
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-02-08 17:07:46 -0700 |
| HMDB ID |
HMDB00024 |
| Secondary Accession Numbers |
None |
| Metabolite Identification |
|---|
| Common Name |
3-O-Sulfogalactosylceramide (d18:1/24:0) |
| 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). |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
|
| Synonyms |
- 3'-O-Sulphogalactosylceramide
- 3-O-Sulfo-beta-D-galactosylceramide
- 3-O-Sulfo-beta-delta-galactosylceramide
- 3-O-Sulfogalactosylceramide
- 3-O-Sulphogalactosylceramide
- Cerebroside 3-sulfate
- Cerebroside 3-sulphate
- Galactosylceramide-sulfate
- Galactosylceramide-sulphate
- Galactosylceramidesulfate
- Galactosylceramidesulphate
- N-[(1S,2R,3E)-2-Hydroxy-1-[[(3-O-sulfo-b-D-galactopyranosyl)oxy]methyl]-3-heptadecen-1-yl]-Tetracosanamide
- N-[(1S,2R,3E)-2-Hydroxy-1-[[(3-O-sulfo-b-D-galactopyranosyl)oxy]methyl]-3-heptadecenyl]-Tetracosanamide
- N-[(1S,2R,3E)-2-Hydroxy-1-[[(3-O-sulfo-beta-delta-galactopyranosyl)oxy]methyl]-3-heptadecen-1-yl]-Tetracosanamide
- N-[(1S,2R,3E)-2-Hydroxy-1-[[(3-O-sulfo-beta-delta-galactopyranosyl)oxy]methyl]-3-heptadecenyl]-Tetracosanamide
- Sulfatide
- Sulfatide (d18:1/24:0)
- [R-[R*,S*-(E)]]-N-[2-hydroxy-1-[[(3-O-sulfo-b-D-galactopyranosyl)oxy]methyl]-3-heptadecenyl]-Tetracosanamide
- [R-[R*,S*-(E)]]-N-[2-hydroxy-1-[[(3-O-sulfo-beta-delta-galactopyranosyl)oxy]methyl]-3-heptadecenyl]-Tetracosanamide
|
| Chemical Formula |
C48H93NO11S |
| Average Molecular Weight |
892.317 |
| Monoisotopic Molecular Weight |
891.646933513 |
| 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 Number |
151122-71-3 |
| 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 Key |
MEAZTWJVOWHKJM-CIAPRIGGSA-N |
| Chemical Taxonomy |
|---|
| Kingdom |
Organic Compounds |
| Super Class |
Lipids |
| Class |
Sphingolipids |
| Sub Class |
Sulfatides |
| 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 Parent |
Sulfatides |
| Ontology |
|---|
| Status |
Detected and Quantified |
| Origin |
|
| 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 |
|---|
| State |
Solid |
| Experimental Properties |
| Property |
Value |
Reference |
| Melting Point |
Not Available |
Not Available |
| Boiling Point |
Not Available |
Not Available |
| Water Solubility |
Not Available |
Not Available |
| LogP |
Not Available |
Not Available |
|
| Predicted Properties |
|
| Spectra |
|---|
|
Not Available
|
| Biological Properties |
|---|
| Cellular Locations |
- Extracellular
- Membrane
- Lysosome
|
| Biofluid Locations |
|
| Tissue Location |
|
| Pathways |
|
| Normal Concentrations |
|---|
|
| Urine |
Detected and Quantified |
|
0.205 (0.07-0.34) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal
|
|
|
| Abnormal Concentrations |
|---|
|
| Urine |
Detected and Quantified |
|
12.7 (1.2-24.2) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Metachromatic 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)
|
| External Links |
|---|
| DrugBank ID |
Not Available |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB021878 |
| KNApSAcK ID |
Not Available |
| Chemspider ID |
4953605 |
| KEGG Compound ID |
C06125 |
| BioCyc ID |
GALACTOSYLCERAMIDE-SULFATE |
| BiGG ID |
Not Available |
| Wikipedia Link |
Sulfatide |
| NuGOwiki Link |
HMDB00024 |
| Metagene Link |
HMDB00024 |
| METLIN ID |
5096 |
| PubChem Compound |
6451121 |
| PDB ID |
Not Available |
| ChEBI ID |
60361 |
| References |
|---|
| 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. |
| Material Safety Data Sheet (MSDS) |
Not Available
|
| 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] Nippon 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
|
| Enzymes |
|---|
| Name: |
Galactocerebrosidase
|
| Reactions: |
- D-galactosyl-N-acylsphingosine + Water unknown Beta-D-Galactose + N-acylsphingosine
- Galactosylceramide + Water unknown Beta-D-Galactose + N-Acylsphingosine
|
| Gene Name: |
GALC |
| Uniprot ID: |
P54803 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
| Name: |
Galactosylceramide sulfotransferase
|
| Reactions: |
- Phosphoadenosine phosphosulfate + a galactosylceramide unknown Adenosine 3',5'-diphosphate + a galactosylceramidesulfate
- Phosphoadenosine phosphosulfate + monogalactosylalkylacylglycerol unknown Adenosine 3',5'-diphosphate + monogalactosylalkylacylglycerol sulfate
- Phosphoadenosine phosphosulfate + Galactosylceramide unknown Adenosine 3',5'-diphosphate + Sulfatide
- Phosphoadenosine phosphosulfate + Digalactosylceramide unknown Adenosine 3',5'-diphosphate + Digalactosylceramidesulfate
|
| Gene Name: |
GAL3ST1 |
| Uniprot ID: |
Q99999 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
| Name: |
Arylsulfatase A
|
| Reactions: |
- A cerebroside 3-sulfate + Water unknown a cerebroside + Oat gum
- Sulfatide + Water unknown Galactosylceramide + Oat gum
|
| Gene Name: |
ARSA |
| Uniprot ID: |
P15289 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
|
|
|
|
| Name: |
Sphingomyelin phosphodiesterase
|
| Reactions: |
- Sphingomyelin + Water unknown N-acylsphingosine + Phosphorylcholine
- Sphingomyelin + Water unknown N-Acylsphingosine + Phosphorylcholine
|
| Gene Name: |
SMPD1 |
| Uniprot ID: |
P17405 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
| Name: |
Ceramide glucosyltransferase
|
| Reactions: |
- Uridine diphosphate glucose + N-acylsphingosine unknown Uridine 5'-diphosphate + D-glucosyl-N-acylsphingosine
- Uridine diphosphate glucose + N-Acylsphingosine unknown Uridine 5'-diphosphate + beta-D-Glucosyl-(1<->1)-ceramide
|
| Gene Name: |
UGCG |
| Uniprot ID: |
Q16739 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
| Name: |
Lactosylceramide 4-alpha-galactosyltransferase
|
| Reactions: |
- Uridine diphosphategalactose + beta-D-galactosyl-(1->4)-D-glucosyl-(1<->1)-ceramide unknown Uridine 5'-diphosphate + alpha-D-galactosyl-(1->4)-beta-D-galactosyl-(1->4)-D-glucosyl-(1<->1)-ceramide
- UDP-D-galactose + Lactosylceramide unknown UDP + Globotriaosylceramide
|
| Gene Name: |
A4GALT |
| Uniprot ID: |
Q9NPC4 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
| Name: |
Phosphatidylinositol N-acetylglucosaminyltransferase subunit A
|
| Reactions: |
- Uridine diphosphate-N-acetylglucosamine + 1-phosphatidyl-1D-myo-inositol unknown Uridine 5'-diphosphate + 6-(N-acetyl-alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol
- UDP-N-acetyl-D-glucosamine + 1-Phosphatidyl-D-myo-inositol unknown UDP +
|
| Gene Name: |
PIGA |
| Uniprot ID: |
P37287 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
|
|
|
|
| Name: |
Acid ceramidase
|
| Reactions: |
- N-acylsphingosine + Water unknown a carboxylate + Sphingosine
- N-Acylsphingosine + Water unknown Fatty acid + Sphingosine
|
| Gene Name: |
ASAH1 |
| Uniprot ID: |
Q13510 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
| Name: |
Sphingomyelin phosphodiesterase 2
|
| Reactions: |
- Sphingomyelin + Water unknown N-acylsphingosine + Phosphorylcholine
- Sphingomyelin + Water unknown N-Acylsphingosine + Phosphorylcholine
|
| Gene Name: |
SMPD2 |
| Uniprot ID: |
O60906 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
|
|
|
|
|
|
| Name: |
Glucosylceramidase
|
| Reactions: |
- D-glucosyl-N-acylsphingosine + Water unknown D-Glucose + N-acylsphingosine
- beta-D-Glucosyl-(1<->1)-ceramide + Water unknown D-Glucose + N-Acylsphingosine
|
| Gene Name: |
GBA |
| Uniprot ID: |
P04062 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
|
|
|
|
|
|
| Name: |
Ceramide synthase 1
|
| Reactions: |
- Acyl-CoA + Sphingosine unknown Coenzyme A + N-Acylsphingosine
- Acyl-CoA + Sphinganine unknown Coenzyme A + Dihydroceramide
|
| Gene Name: |
CERS1 |
| Uniprot ID: |
P27544 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
|
|
|
|
|
|
|
|
|
|
| Name: |
Lactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferase
|
| Reactions: |
- Uridine diphosphate-N-acetylglucosamine + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide unknown Uridine 5'-diphosphate + N-acetyl-beta-D-glucosaminyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
- UDP-N-acetyl-D-glucosamine + Lactosylceramide unknown UDP + Lc3Cer
|
| Gene Name: |
B3GNT5 |
| Uniprot ID: |
Q9BYG0 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
| Name: |
Sphingomyelin phosphodiesterase 3
|
| Reactions: |
- Sphingomyelin + Water unknown N-acylsphingosine + Phosphorylcholine
- Sphingomyelin + Water unknown N-Acylsphingosine + Phosphorylcholine
|
| Gene Name: |
SMPD3 |
| Uniprot ID: |
Q9NY59 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
| Name: |
Beta-1,4-galactosyltransferase 6
|
| Reactions: |
- UDP-L-rhamnose + Glucosylceramide unknown Uridine 5'-diphosphate + Lactosylceramide (d18:1/24:0)
- Uridine diphosphategalactose + beta-D-glucosyl-(1<->1)-ceramide unknown Uridine 5'-diphosphate + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
- beta-D-Glucosyl-(1<->1)-ceramide + Uridine diphosphategalactose unknown beta-D-Galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide + Uridine 5'-diphosphate
- Glucocerebroside + UDP-D-galactose unknown Lactosylceramide + UDP
|
| Gene Name: |
B4GALT6 |
| Uniprot ID: |
Q9UBX8 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
| Name: |
Sphingomyelin phosphodiesterase 4
|
| Reactions: |
- Sphingomyelin + Water unknown N-acylsphingosine + Phosphorylcholine
- Sphingomyelin + Water unknown N-Acylsphingosine + Phosphorylcholine
|
| Gene Name: |
SMPD4 |
| Uniprot ID: |
Q9NXE4 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
|
|
| Name: |
Alkaline ceramidase 2
|
| Reactions: |
- N-acylsphingosine + Water unknown a carboxylate + Sphingosine
- N-Acylsphingosine + Water unknown Fatty acid + Sphingosine
- Dihydroceramide + Water unknown Fatty acid + Sphinganine
- Phytoceramide + Water unknown Fatty acid + Phytosphingosine
|
| Gene Name: |
ACER2 |
| Uniprot ID: |
Q5QJU3 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
| Name: |
Neutral ceramidase
|
| Reactions: |
- N-acylsphingosine + Water unknown a carboxylate + Sphingosine
|
| Gene Name: |
ASAH2 |
| Uniprot ID: |
Q9NR71 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
| Name: |
Alkaline ceramidase 1
|
| Reactions: |
- N-acylsphingosine + Water unknown a carboxylate + Sphingosine
- N-Acylsphingosine + Water unknown Fatty acid + Sphingosine
- Dihydroceramide + Water unknown Fatty acid + Sphinganine
- Phytoceramide + Water unknown Fatty acid + Phytosphingosine
|
| Gene Name: |
ACER1 |
| Uniprot ID: |
Q8TDN7 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
| Name: |
Ceramide synthase 5
|
| Reactions: |
- Acyl-CoA + Sphingosine unknown Coenzyme A + N-Acylsphingosine
- Acyl-CoA + Sphinganine unknown Coenzyme A + Dihydroceramide
|
| Gene Name: |
CERS5 |
| Uniprot ID: |
Q8N5B7 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
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|
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