| Record Information |
|---|
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-02-08 17:13:40 -0700 |
| HMDB ID |
HMDB04961 |
| Secondary Accession Numbers |
None |
| Metabolite Identification |
|---|
| Common Name |
Tetrahexosylceramide (d18:1/18:0) |
| Description |
Tetrahexosylceramide (d18:1/18:0) is a glycosphingolipid (ceramide and oligosaccharide)or oligoglycosylceramide with one or more sialic acids (i.e. n-acetylneuraminic acid) linked on the sugar chain. Tetrahexosylceramide contains a tetrasaccharide moiety bound in glycosidic linkage to the hydroxyl group of ceramide as the polar head group. It is a component the cell plasma membrane which modulates cell signal transduction events. Gangliosides have been found to be highly important in immunology. Ganglioside Gb4 carries a net-negative charge at pH 7.0 and is acidic. Gangliosides can amount to 6% of the weight of lipids from brain, but they are found at low levels in all animal tissues.Globosides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Globosides are cerebrosides that contain additional carbohydrates, predominantly galactose, glucose or GalNAc. Lactosyl ceramide is a globoside found in erythrocyte plasma membranes. Globotriaosylceramide (also called ceramide trihexoside) contains glucose and two moles of galactose and accumulates, primarily in the kidneys, of patients suffering from Fabry disease. |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
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| Synonyms |
- CQH
- Cytolipin K
- GalNAc-beta1->3Gal-alpha1->3Gal-beta1->4Glc-beta1->1'Cer
- Ganglioside Gb4
- Ganglioside GL 4
- Gb4
- Gb4Cer
- Globo-N-tetraosylceramide
- Globosidate
- Globoside
- Globoside Gb 4
- Globoside GL 4
- Globoside I
- Globosidic acid
- Globotetraosylceramide
- IGb4Cer
- N-Acetyl-D-galactosaminyl-1,3-D-galactosyl-1,4-D-galactosyl-1,4-D-glucosylceramide
- N-Acetyl-delta-galactosaminyl-1,3-delta-galactosyl-1,4-delta-galactosyl-1,4-delta-glucosylceramide
- N-Acetyl-galactosaminyl-1,3-D-galactosyl-1,4-D-galactosyl-1,4-D-glucosylceramide
- N-Acetyl-galactosaminyl-1,3-delta-galactosyl-1,4-delta-galactosyl-1,4-delta-glucosylceramide
- Parvovirus B19 receptor
|
| Chemical Formula |
C62H114N2O23 |
| Average Molecular Weight |
1255.5682 |
| Monoisotopic Molecular Weight |
1254.781237964 |
| IUPAC Name |
N-[(2S,3R,4E)-1-{[(2R,4R,5S,6R)-5-{[(2S,3R,4R,5R,6R)-5-{[(2R,3R,4S,5S,6R)-4-{[(2S,3R,4R,5R,6R)-3-acetamido-4,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3-hydroxyoctadec-4-en-2-yl]octadecanamide |
| Traditional IUPAC Name |
N-[(2S,3R,4E)-1-{[(2R,4R,5S,6R)-5-{[(2S,3R,4R,5R,6R)-5-{[(2R,3R,4S,5S,6R)-4-{[(2S,3R,4R,5R,6R)-3-acetamido-4,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,4-dihydr |
| CAS Registry Number |
11034-93-8 |
| SMILES |
CCCCCCCCCCCCCCCCCC(=O)N[C@@H](CO[C@@H]1O[C@H](CO)[C@@H](O[C@@H]2O[C@H](CO)[C@H](O[C@H]3O[C@H](CO)[C@H](O)[C@H](O[C@@H]4O[C@H](CO)[C@H](O)[C@H](O)[C@H]4NC(C)=O)[C@H]3O)[C@H](O)[C@H]2O)[C@H](O)C1O)[C@H](O)\C=C\CCCCCCCCCCCCC |
| InChI Identifier |
InChI=1S/C62H114N2O23/c1-4-6-8-10-12-14-16-18-19-21-23-25-27-29-31-33-46(71)64-40(41(70)32-30-28-26-24-22-20-17-15-13-11-9-7-5-2)38-80-60-53(77)51(75)56(44(36-67)83-60)85-61-54(78)52(76)57(45(37-68)84-61)86-62-55(79)58(49(73)43(35-66)82-62)87-59-47(63-39(3)69)50(74)48(72)42(34-65)81-59/h30,32,40-45,47-62,65-68,70,72-79H,4-29,31,33-38H2,1-3H3,(H,63,69)(H,64,71)/b32-30+/t40-,41+,42+,43+,44+,45+,47+,48-,49-,50+,51+,52+,53?,54+,55+,56+,57-,58-,59-,60+,61-,62+/m0/s1 |
| InChI Key |
WQRPYKCGVLWFGH-FRDPBZGNSA-N |
| Chemical Taxonomy |
|---|
| Kingdom |
Organic Compounds |
| Super Class |
Lipids |
| Class |
Sphingolipids |
| Sub Class |
Neutral Glycosphingolipids |
| Other Descriptors |
- Aliphatic Heteropolycyclic Compounds
|
| Substituents |
- 1,2 Diol
- Acetal
- Alkyl Glycoside
- Allyl Alcohol
- Carboxamide Group
- Ceramide
- Fatty Acyl Glycoside
- Glucosamine
- Glycosyl Compound
- Hexose Tetrasaccharide
- Mannosamine
- N Acyl Amine
- O Glycosyl Compound
- Oxane
- Primary Alcohol
- Saccharide
- Secondary Alcohol
- Secondary Carboxylic Acid Amide
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| Direct Parent |
GalNAcb1-3Gala1-4Galb1-4Glc- (Globo series) |
| Ontology |
|---|
| Status |
Expected and Not Quantified |
| Origin |
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| Biofunction |
- Cell signaling
- Fuel and energy storage
- Fuel or energy source
- Membrane integrity/stability
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| Application |
- Nutrients
- Stabilizers
- Surfactants and Emulsifiers
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| Cellular locations |
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| Physical Properties |
|---|
| State |
Solid |
| Experimental Properties |
| Property |
Value |
Reference |
| Melting Point |
Not Available |
Not Available |
| Boiling Point |
Not Available |
Not Available |
| Water Solubility |
Insoluble |
Not Available |
| LogP |
Not Available |
Not Available |
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| Predicted Properties |
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| Spectra |
|---|
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Not Available
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| Biological Properties |
|---|
| Cellular Locations |
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| Biofluid Locations |
Not Available
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| Tissue Location |
- Fibroblasts
- Erythrocyte
- Kidney
- Liver
- Brain
- Nerve Cells
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| Pathways |
Not Available
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| Normal Concentrations |
|---|
|
Not Available |
| Abnormal Concentrations |
|---|
|
Not Available |
| Associated Disorders and Diseases |
|---|
| Disease References |
None |
| Associated OMIM IDs |
None |
| External Links |
|---|
| DrugBank ID |
Not Available |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB023548 |
| KNApSAcK ID |
Not Available |
| Chemspider ID |
16744946  |
| KEGG Compound ID |
C03272 |
| BioCyc ID |
N-ACETYL-D-GALACTOSAMINYL-13-D-GALACT |
| BiGG ID |
Not Available |
| Wikipedia Link |
CQH |
| NuGOwiki Link |
HMDB04961 |
| Metagene Link |
HMDB04961 |
| METLIN ID |
7214 |
| PubChem Compound |
20057346 |
| PDB ID |
Not Available |
| ChEBI ID |
Not Available |
| References |
|---|
| Synthesis Reference |
Not Available |
| Material Safety Data Sheet (MSDS) |
Not Available
|
| General References |
- Kannagi R, Fukuda MN, Hakomori S: A new glycolipid antigen isolated from human erythrocyte membranes reacting with antibodies directed to globo-N-tetraosylceramide (globoside). J Biol Chem. 1982 Apr 25;257(8):4438-42.
Pubmed: 7068643
- Kunishita T, Taketomi T: Sphingomyelin storage in a patient with myoclonus epilepsy as a main clinical symptom -- a varient in Niemann-Pick disease type C. Jpn J Exp Med. 1979 Apr;49(2):151-6.
Pubmed: 113605
- Zdebska E, Mendek-Czajkowska E, Ploski R, Woeniewicz B, Koscielak J: Heterozygosity of CDAN II (HEMPAS) gene may be detected by the analysis of erythrocyte membrane glycoconjugates from healthy carriers. Haematologica. 2002 Feb;87(2):126-30.
Pubmed: 11836161
- Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31.
Pubmed: 178813
- Hansson GC, Wazniowska K, Rock JA, Ness PM, Kickler TS, Shirey RS, Niebyl JR, Zopf D: The glycosphingolipid composition of the placenta of a blood group P fetus delivered by a blood group Pk1 woman and analysis of the anti-globoside antibodies found in maternal serum. Arch Biochem Biophys. 1988 Jan;260(1):168-76.
Pubmed: 3341739
- Tillack TW, Allietta M, Moran RE, Young WW Jr: Localization of globoside and Forssman glycolipids on erythrocyte membranes. Biochim Biophys Acta. 1983 Aug 24;733(1):15-24.
Pubmed: 6603868
- Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30.
Pubmed: 12829005
- Choudhury A, Dominguez M, Puri V, Sharma DK, Narita K, Wheatley CL, Marks DL, Pagano RE: Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells. J Clin Invest. 2002 Jun;109(12):1541-50.
Pubmed: 12070301
- Ledvinova J, Poupetova H, Hanackova A, Pisacka M, Elleder M: Blood group B glycosphingolipids in alpha-galactosidase deficiency (Fabry disease): influence of secretor status. Biochim Biophys Acta. 1997 Apr 1;1345(2):180-7.
Pubmed: 9106497
- Marcus DM, Naiki M, Kundu SK: Abnormalities in the glycosphingolipid content of human Pk and p erythrocytes. Proc Natl Acad Sci U S A. 1976 Sep;73(9):3263-7.
Pubmed: 1067617
- Kundu SK, Steane SM, Bloom JE, Marcus DM: Abnormal glycolipid composition of erythrocytes with a weak P antigen. Vox Sang. 1978 Sep;35(3):160-7.
Pubmed: 676244
- Ogawa-Goto K, Ohta Y, Kubota K, Funamoto N, Abe T, Taki T, Nagashima K: Glycosphingolipids of human peripheral nervous system myelins isolated from cauda equina. J Neurochem. 1993 Oct;61(4):1398-403.
Pubmed: 7690848
- Tatematsu M, Imaida K, Ito N, Togari H, Suzuki Y, Ogiu T: Sandhoff disease. Acta Pathol Jpn. 1981 May;31(3):503-12.
Pubmed: 7270152
- Zdebska E, Anselstetter V, Pacuszka T, Krauze R, Chelstowska A, Heimpel H, Koscielak J: Glycolipids and glycopeptides of red cell membranes in congenital dyserythropoietic anaemia type II (CDA II). Br J Haematol. 1987 Jul;66(3):385-91.
Pubmed: 3620357
- Snyder PD Jr, Krivit W, Sweeley CC: Generalized accumulation of neutral glycosphingolipids with G M2 ganglioside accumulation in the brain. J Lipid Res. 1972 Jan;13(1):128-36.
Pubmed: 5059190
- Leffler H, Lomberg H, Gotschlich E, Hagberg L, Jodal U, Korhonen T, Samuelsson BE, Schoolnik G, Svanborg-Eden C: Chemical and clinical studies on the interaction of Escherichia coli with host glycolipid receptors in urinary tract infection. Scand J Infect Dis Suppl. 1982;33:46-51.
Pubmed: 6127802
- Kijimoto-Ochiai S, Naiki M, Makita A: Defects of glycosyltransferase activities in human fibroblasts of Pk and p blood group phenotypes. Proc Natl Acad Sci U S A. 1977 Dec;74(12):5407-10.
Pubmed: 271963
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| Enzymes |
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| Name: |
Galactocerebrosidase
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| Reactions: |
- D-galactosyl-N-acylsphingosine + H2O = D-galactose + N-acylsphingosine [RN:R03617]
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| Gene Name: |
GALC |
| Uniprot ID: |
P54803 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Acid ceramidase
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| Reactions: |
- N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
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| Gene Name: |
ASAH1 |
| Uniprot ID: |
Q13510 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Glucosylceramidase
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| Reactions: |
- D-glucosyl-N-acylsphingosine + H2O = D-glucose + N-acylsphingosine [RN:R01498]
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| Gene Name: |
GBA |
| Uniprot ID: |
P04062 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Alkaline ceramidase 2
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| Reactions: |
- N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
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| Gene Name: |
ACER2 |
| Uniprot ID: |
Q5QJU3 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Neutral ceramidase
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| Reactions: |
- N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
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| Gene Name: |
ASAH2 |
| Uniprot ID: |
Q9NR71 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Alkaline ceramidase 1
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| Reactions: |
- N-acylsphingosine + H2O = a carboxylate + sphingosine [RN:R01493]
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| Gene Name: |
ACER1 |
| Uniprot ID: |
Q8TDN7 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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