| Record Information |
|---|
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-02-08 17:13:29 -0700 |
| HMDB ID |
HMDB04884 |
| Secondary Accession Numbers |
|
| Metabolite Identification |
|---|
| Common Name |
Trihexosylceramide (d18:1/26:1(17Z)) |
| Description |
Trihexosylceramide is a glycosphingolipid which contains a trisaccharide (galactose-galactose-glucose) moiety bound in glycosidic linkage to the hydroxyl group of ceramide as the polar head group. It accumulates in tissue due to a defect in ceramide trihexosidase and is the cause of angiokeratoma corporis diffusum (Fabry disease). Although normal human colonic epithelial cells lack the glycosphingolipid globotriaosylceramide (Gb(3)), this molecule is highly expressed in metastatic colon cancer (PubMed ID 16365318 ). |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
|
| Synonyms |
- Ceramide trihexoside
- D-Galactosyl-1,4-D-galactosyl-1,4-D-glucosylceramide
- delta-Galactosyl-1,4-delta-galactosyl-1,4-delta-glucosylceramide
- Fabry glycolipid
- Gal-alpha1->4gal-beta1->4glc-beta1->1'cer
- Gal-alpha1->4Gal-beta1->4Glc-beta1->1'Cer(d18:1/26:1
- Gal-alpha1->4laccer
- Ganglioside GL3
- Gb3
- Globo-N-triaosylceramide
- Globotriaosylceramide
- Globotriglycosylceramide
- Globotriosylceramide
- Shiga toxin receptor
|
| Chemical Formula |
C62H117NO18 |
| Average Molecular Weight |
1164.5883 |
| Monoisotopic Molecular Weight |
1163.827065945 |
| IUPAC Name |
N-[(4E)-1-[(5-{[3,4-dihydroxy-6-(hydroxymethyl)-5-{[3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl)oxy]-3-hydroxyoctadec-4-en-2-yl]hexacosanamide |
| Traditional IUPAC Name |
N-[(4E)-1-[(5-{[3,4-dihydroxy-6-(hydroxymethyl)-5-{[3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl)oxy]-3-hydroxyoctadec-4-en-2-yl]hexacosanamide |
| CAS Registry Number |
71965-57-6 |
| SMILES |
CCCCCCCCCCCCCCCCCCCCCCCCCC(=O)NC(COC1OC(CO)C(OC2OC(CO)C(OC3OC(CO)C(O)C(O)C3O)C(O)C2O)C(O)C1O)C(O)\C=C\CCCCCCCCCCCCC |
| InChI Identifier |
InChI=1S/C62H117NO18/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-23-24-25-26-28-30-32-34-36-38-40-50(68)63-45(46(67)39-37-35-33-31-29-27-16-14-12-10-8-6-4-2)44-76-60-56(74)53(71)58(48(42-65)78-60)81-62-57(75)54(72)59(49(43-66)79-62)80-61-55(73)52(70)51(69)47(41-64)77-61/h37,39,45-49,51-62,64-67,69-75H,3-36,38,40-44H2,1-2H3,(H,63,68)/b39-37+ |
| InChI Key |
YWHNDSBMLSKIRV-NHFVQZBWSA-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
- Glycosyl Compound
- Hexose Trisaccharide
- 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 |
Detected and Quantified |
| Origin |
|
| Biofunction |
- Cell signaling
- Component of Globoside metabolism
- Fuel and energy storage
- Fuel or energy source
- Membrane integrity/stability
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| Application |
- Nutrients
- Stabilizers
- Surfactants and Emulsifiers
|
| Cellular locations |
|
| 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 |
|
| Spectra |
|---|
|
Not Available
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| Biological Properties |
|---|
| Cellular Locations |
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| Biofluid Locations |
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| Tissue Location |
- Kidney
- Liver
- Brain
- Skin
- Nerve Cells
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| Pathways |
Not Available
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| Normal Concentrations |
|---|
|
| Blood |
Detected and Quantified |
|
2.0 +/- 1.0 uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.0001842-0.0003947 umol/mmol creatinine |
Adult (>18 years old) |
Not Specified |
Normal |
Not Available |
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| Abnormal Concentrations |
|---|
|
| Blood |
Detected and Quantified |
|
1.0 (0.6-1.7) uM |
Adult (>18 years old) |
Both |
Abetalipoproteinemia |
Not Available |
| Blood |
Detected and Quantified |
|
0.9 (0.9-1.0) uM |
Adult (>18 years old) |
Both |
Hypobetalipoproteinemia |
Not Available |
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| Associated Disorders and Diseases |
|---|
| Disease References |
| Abetalipoproteinemia |
|---|
- 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
|
| Hypobetalipoproteinemia |
|---|
- 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
|
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| Associated OMIM IDs |
|
| External Links |
|---|
| DrugBank ID |
Not Available |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB023477 |
| KNApSAcK ID |
Not Available |
| Chemspider ID |
16744897 |
| KEGG Compound ID |
C04737 |
| BioCyc ID |
D-GALACTOSYL-14-D-GALACTOSYL-14-D- |
| BiGG ID |
Not Available |
| Wikipedia Link |
Not Available |
| NuGOwiki Link |
HMDB04884 |
| Metagene Link |
HMDB04884 |
| METLIN ID |
7141 |
| PubChem Compound |
20057319 |
| PDB ID |
Not Available |
| ChEBI ID |
Not Available |
| References |
|---|
| Synthesis Reference |
Not Available |
| Material Safety Data Sheet (MSDS) |
Not Available
|
| General References |
- Hara A, Kitazawa N, Taketomi T: Abnormalities of glycosphingolipids in mucopolysaccharidosis type III B. J Lipid Res. 1984 Feb;25(2):175-84.
Pubmed: 6423755
- 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
- Thurberg BL, Randolph Byers H, Granter SR, Phelps RG, Gordon RE, O'Callaghan M: Monitoring the 3-year efficacy of enzyme replacement therapy in fabry disease by repeated skin biopsies. J Invest Dermatol. 2004 Apr;122(4):900-8.
Pubmed: 15102080
- 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
- Thurberg BL, Rennke H, Colvin RB, Dikman S, Gordon RE, Collins AB, Desnick RJ, O'Callaghan M: Globotriaosylceramide accumulation in the Fabry kidney is cleared from multiple cell types after enzyme replacement therapy. Kidney Int. 2002 Dec;62(6):1933-46.
Pubmed: 12427118
- Kovbasnjuk O, Mourtazina R, Baibakov B, Wang T, Elowsky C, Choti MA, Kane A, Donowitz M: The glycosphingolipid globotriaosylceramide in the metastatic transformation of colon cancer. Proc Natl Acad Sci U S A. 2005 Dec 27;102(52):19087-92. Epub 2005 Dec 19.
Pubmed: 16365318
- Roder B, Dabrowski J, Dabrowski U, Egge H, Peter-Katalinic J, Schwarzmann G, Sandhoff K: The determination of phytosphingosine-containing globotriaosylceramide from human kidney in the presence of lactosylceramide. Chem Phys Lipids. 1990 Mar;53(1):85-9.
Pubmed: 2162261
- Eng CM, Guffon N, Wilcox WR, Germain DP, Lee P, Waldek S, Caplan L, Linthorst GE, Desnick RJ: Safety and efficacy of recombinant human alpha-galactosidase A--replacement therapy in Fabry's disease. N Engl J Med. 2001 Jul 5;345(1):9-16.
Pubmed: 11439963
- 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
- Abe A, Wild SR, Lee WL, Shayman JA: Agents for the treatment of glycosphingolipid storage disorders. Curr Drug Metab. 2001 Sep;2(3):331-8.
Pubmed: 11513334
- Mignani R, Cagnoli L: Enzyme replacement therapy in Fabry's disease: recent advances and clinical applications. J Nephrol. 2004 May-Jun;17(3):354-63.
Pubmed: 15365954
- Kanekura T, Fukushige T, Kanda A, Tsuyama S, Murata F, Sakuraba H, Kanzaki T: Immunoelectron-microscopic detection of globotriaosylceramide accumulated in the skin of patients with Fabry disease. Br J Dermatol. 2005 Sep;153(3):544-8.
Pubmed: 16120140
- Li SC, Kundu SK, Degasperi R, Li YT: Accumulation of globotriaosylceramide in a case of leiomyosarcoma. Biochem J. 1986 Dec 15;240(3):925-7.
Pubmed: 3827883
- Wilcox WR, Banikazemi M, Guffon N, Waldek S, Lee P, Linthorst GE, Desnick RJ, Germain DP: Long-term safety and efficacy of enzyme replacement therapy for Fabry disease. Am J Hum Genet. 2004 Jul;75(1):65-74. Epub 2004 May 20.
Pubmed: 15154115
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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: |
Alpha-galactosidase A
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| Reactions: |
- Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids ALL_REAC (other) R01101 R01103 R01104 R01194 R01329 R02926 R03618 R03634 R04019 R04470 R05549 R05961(G) R06070(G) R06091(G) R06093(G) R06094(G) R06096(G) R06142(G) R06152(G)
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| Gene Name: |
GLA |
| Uniprot ID: |
P06280 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Beta-hexosaminidase subunit beta
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| Reactions: |
- Hydrolysis of terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides ALL_REAC (other) R00022 R03492 R04184 R04586 R05963(G) R06001(G) R06004(G) R06141(G) R07809(G) R07810(G)
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| Gene Name: |
HEXB |
| Uniprot ID: |
P07686 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Beta-hexosaminidase subunit alpha
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| Reactions: |
- Hydrolysis of terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides ALL_REAC (other) R00022 R03492 R04184 R04586 R05963(G) R06001(G) R06004(G) R06141(G) R07809(G) R07810(G)
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| Gene Name: |
HEXA |
| Uniprot ID: |
P06865 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Lactosylceramide 4-alpha-galactosyltransferase
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| Reactions: |
- UDP-galactose + beta-D-galactosyl-(1->4)-D-glucosyl-(11)-ceramide = UDP + alpha-D-galactosyl-(1->4)-beta-D-galactosyl-(1->4)-D-glucosyl-(11)- ceramide [RN:R03486 R05960]
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| Gene Name: |
A4GALT |
| Uniprot ID: |
Q9NPC4 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
UDP-GalNAc:beta-1,3-N-acetylgalactosaminyltransferase 1
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| Reactions: |
- UDP-N-acetyl-D-galactosamine + alpha-D-galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl- (11)-ceramide = UDP + N-acetyl-beta-D-galactosaminyl-(1->3)-alpha-D-galactosyl-(1->4)- beta-D-galactosyl-(1->4)-beta-D-glucosyl-(11)-ceramide [RN:R05631 R05962]
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| Gene Name: |
B3GALNT1 |
| Uniprot ID: |
O75752 |
| 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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