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Human Metabolome Database Version 3.5

Showing metabocard for Trihexosylceramide (d18:1/25:0) (HMDB04887)

• Identification
• Taxonomy
• Ontology
• Physical properties
• Spectra
• Biological properties
• Concentrations
• Links
• References

enzymes (64)

• Blood
• Urine

Record Information
Version	3.5
Creation Date	2005-11-16 08:48:42 -0700
Update Date	2013-02-08 17:13:30 -0700
HMDB ID	HMDB04887
Secondary Accession Numbers	None
Metabolite Identification
Common Name	Trihexosylceramide (d18:1/25:0)
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->4laccer Ganglioside GL3 Gb3 Globo-N-triaosylceramide Globotriaosylceramide Globotriglycosylceramide Globotriosylceramide Shiga toxin receptor
Chemical Formula	C61H115NO18
Average Molecular Weight	1150.5617
Monoisotopic Molecular Weight	1149.811415881
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]pentacosanamide
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]pentacosanamide
CAS Registry Number	71965-57-6
SMILES	CCCCCCCCCCCCCCCCCCCCCCCCC(=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/C61H115NO18/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-23-24-25-27-29-31-33-35-37-39-49(67)62-44(45(66)38-36-34-32-30-28-26-16-14-12-10-8-6-4-2)43-75-59-55(73)52(70)57(47(41-64)77-59)80-61-56(74)53(71)58(48(42-65)78-61)79-60-54(72)51(69)50(68)46(40-63)76-60/h36,38,44-48,50-61,63-66,68-74H,3-35,37,39-43H2,1-2H3,(H,62,67)/b38-36+
InChI Key	KTCNLGUVWRZEDW-BSKJHSHCSA-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
Direct Parent	GalNAcb1-3Gala1-4Galb1-4Glc- (Globo series)
Ontology
Status	Detected and Quantified
Origin	Endogenous Food
Biofunction	Cell signaling Component of Globoside metabolism Fuel and energy storage Fuel or energy source Membrane integrity/stability
Application	Nutrients Stabilizers Surfactants and Emulsifiers
Cellular locations	Extracellular Membrane
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
Predicted Properties	Property Value Source Water Solubility 0.0096 g/L ALOGPS LogP 6.02 ALOGPS LogP 9.55 ChemAxon LogS -5.08 ALOGPS pKa (strongest acidic) 11.76 ChemAxon pKa (strongest basic) -3.6 ChemAxon Hydrogen Acceptor Count 18 ChemAxon Hydrogen Donor Count 12 ChemAxon Polar Surface Area 307.01 A2 ChemAxon Rotatable Bond Count 48 ChemAxon Refractivity 304.42 ChemAxon Polarizability 138.14 ChemAxon Formal Charge 0 ChemAxon Physiological Charge 0 ChemAxon
Spectra
	Not Available
Biological Properties
Cellular Locations	Extracellular Membrane
Biofluid Locations	Urine
Tissue Location	Kidney Liver Brain Skin Nerve Cells
Pathways	Not Available
Normal Concentrations
	Biofluid Status Value Age Sex Condition Reference Urine Detected and Quantified 0.0001842-0.0003947 umol/mmol creatinine Adult (>18 years old) Not Specified Normal 9106497
Abnormal Concentrations
	Not Available
Associated Disorders and Diseases
Disease References	None
Associated OMIM IDs	None
External Links
DrugBank ID	Not Available
DrugBank Metabolite ID	Not Available
Phenol Explorer Compound ID	Not Available
Phenol Explorer Metabolite ID	Not Available
FoodDB ID	FDB023479
KNApSAcK ID	Not Available
Chemspider ID	16744899
KEGG Compound ID	C04737
BioCyc ID	D-GALACTOSYL-14-D-GALACTOSYL-14-D-
BiGG ID	Not Available
Wikipedia Link	Not Available
NuGOwiki Link	HMDB04887
Metagene Link	HMDB04887
METLIN ID	7144
PubChem Compound	20057320
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

---

Enzymes
Name: Galactocerebrosidase Reactions: Not Available Gene Name: GALC Uniprot ID: P54803 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Globoside alpha-1,3-N-acetylgalactosaminyltransferase 1 Reactions: Not Available Gene Name: GBGT1 Uniprot ID: Q8N5D6 Protein Sequence: FASTA Gene Sequence: FASTA
Name: N-acetylglucosaminyl-phosphatidylinositol de-N-acetylase Reactions: Not Available Gene Name: PIGL Uniprot ID: Q9Y2B2 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Alpha-galactosidase A Reactions: Not Available Gene Name: GLA Uniprot ID: P06280 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Sphingomyelin phosphodiesterase Reactions: Not Available Gene Name: SMPD1 Uniprot ID: P17405 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Ceramide glucosyltransferase Reactions: Not Available Gene Name: UGCG Uniprot ID: Q16739 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Beta-hexosaminidase subunit beta Reactions: Not Available Gene Name: HEXB Uniprot ID: P07686 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Beta-hexosaminidase subunit alpha Reactions: Not Available Gene Name: HEXA Uniprot ID: P06865 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Lactosylceramide 4-alpha-galactosyltransferase Reactions: Not Available Gene Name: A4GALT Uniprot ID: Q9NPC4 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit Q Reactions: Not Available Gene Name: PIGQ Uniprot ID: Q9BRB3 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit A Reactions: Not Available Gene Name: PIGA Uniprot ID: P37287 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit H Reactions: Not Available Gene Name: PIGH Uniprot ID: Q14442 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit P Reactions: Not Available Gene Name: PIGP Uniprot ID: P57054 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit C Reactions: Not Available Gene Name: PIGC Uniprot ID: Q92535 Protein Sequence: FASTA Gene Sequence: FASTA
Name: UDP-GalNAc:beta-1,3-N-acetylgalactosaminyltransferase 1 Reactions: Not Available Gene Name: B3GALNT1 Uniprot ID: O75752 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Acid ceramidase Reactions: Not Available Gene Name: ASAH1 Uniprot ID: Q13510 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Ectonucleotide pyrophosphatase/phosphodiesterase family member 7 Reactions: Not Available Gene Name: ENPP7 Uniprot ID: Q6UWV6 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Sphingomyelin phosphodiesterase 2 Reactions: Not Available Gene Name: SMPD2 Uniprot ID: O60906 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Collagen type IV alpha-3-binding protein Reactions: Not Available Gene Name: COL4A3BP Uniprot ID: Q9Y5P4 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Ganglioside GM2 activator Reactions: Not Available Gene Name: GM2A Uniprot ID: P17900 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glucosylceramidase Reactions: Not Available Gene Name: GBA Uniprot ID: P04062 Protein Sequence: FASTA Gene Sequence: FASTA
Name: T-cell surface glycoprotein CD1e, membrane-associated Reactions: Not Available Gene Name: CD1E Uniprot ID: P15812 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Epididymal secretory protein E1 Reactions: Not Available Gene Name: NPC2 Uniprot ID: P61916 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Ceramide synthase 1 Reactions: Not Available Gene Name: CERS1 Uniprot ID: P27544 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Antigen-presenting glycoprotein CD1d Reactions: Not Available Gene Name: CD1D Uniprot ID: P15813 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI mannosyltransferase 1 Reactions: Not Available Gene Name: PIGM Uniprot ID: Q9H3S5 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol-glycan biosynthesis class W protein Reactions: Not Available Gene Name: PIGW Uniprot ID: Q7Z7B1 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol-glycan biosynthesis class X protein Reactions: Not Available Gene Name: PIGX Uniprot ID: Q8TBF5 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI mannosyltransferase 4 Reactions: Not Available Gene Name: PIGZ Uniprot ID: Q86VD9 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Lactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferase Reactions: Not Available Gene Name: B3GNT5 Uniprot ID: Q9BYG0 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Sphingomyelin phosphodiesterase 3 Reactions: Not Available Gene Name: SMPD3 Uniprot ID: Q9NY59 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Beta-1,3-galactosyltransferase 5 Reactions: Not Available Gene Name: B3GALT5 Uniprot ID: Q9Y2C3 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI mannosyltransferase 3 Reactions: Not Available Gene Name: PIGB Uniprot ID: Q92521 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol-glycan biosynthesis class F protein Reactions: Not Available Gene Name: PIGF Uniprot ID: Q07326 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI ethanolamine phosphate transferase 2 Reactions: Not Available Gene Name: PIGG Uniprot ID: Q5H8A4 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI ethanolamine phosphate transferase 1 Reactions: Not Available Gene Name: PIGN Uniprot ID: O95427 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI ethanolamine phosphate transferase 3 Reactions: Not Available Gene Name: PIGO Uniprot ID: Q8TEQ8 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI transamidase component PIG-S Reactions: Not Available Gene Name: PIGS Uniprot ID: Q96S52 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI transamidase component PIG-T Reactions: Not Available Gene Name: PIGT Uniprot ID: Q969N2 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol glycan anchor biosynthesis class U protein Reactions: Not Available Gene Name: PIGU Uniprot ID: Q9H490 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI mannosyltransferase 2 Reactions: Not Available Gene Name: PIGV Uniprot ID: Q9NUD9 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Phosphatidylinositol N-acetylglucosaminyltransferase subunit Y Reactions: Not Available Gene Name: PIGY Uniprot ID: Q3MUY2 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Pleckstrin homology domain-containing family A member 8 Reactions: Not Available Gene Name: PLEKHA8 Uniprot ID: Q96JA3 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Sphingomyelin phosphodiesterase 4 Reactions: Not Available Gene Name: SMPD4 Uniprot ID: Q9NXE4 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Putative neutral ceramidase C Reactions: Not Available Gene Name: ASAH2C Uniprot ID: P0C7U2 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Alkaline ceramidase 2 Reactions: Not Available Gene Name: ACER2 Uniprot ID: Q5QJU3 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Neutral ceramidase Reactions: Not Available Gene Name: ASAH2 Uniprot ID: Q9NR71 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Non-lysosomal glucosylceramidase Reactions: Not Available Gene Name: GBA2 Uniprot ID: Q9HCG7 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Alkaline ceramidase 1 Reactions: Not Available Gene Name: ACER1 Uniprot ID: Q8TDN7 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Killer cell lectin-like receptor subfamily B member 1 Reactions: Not Available Gene Name: KLRB1 Uniprot ID: Q12918 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Ceramide synthase 5 Reactions: Not Available Gene Name: CERS5 Uniprot ID: Q8N5B7 Protein Sequence: FASTA Gene Sequence: FASTA
Name: ATP-binding cassette sub-family A member 7 Reactions: Not Available Gene Name: ABCA7 Uniprot ID: Q8IZY2 Protein Sequence: FASTA Gene Sequence: FASTA
Name: GPI-anchor transamidase Reactions: Not Available Gene Name: PIGK Uniprot ID: Q92643 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycosylphosphatidylinositol anchor attachment 1 protein Reactions: Not Available Gene Name: GPAA1 Uniprot ID: O43292 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Protein PLEKHA9 Reactions: Not Available Gene Name: PLEKHA9 Uniprot ID: O95397 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycolipid transfer protein domain-containing protein 2 Reactions: Not Available Gene Name: GLTPD2 Uniprot ID: A6NH11 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Putative uncharacterized protein PLEKHA8 Reactions: Not Available Gene Name: PLEKHA8 Uniprot ID: B5MDU3 Protein Sequence: FASTA Gene Sequence: FASTA
Name: T-cell surface glycoprotein CD1a Reactions: Not Available Gene Name: CD1A Uniprot ID: P06126 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycolipid transfer protein domain-containing protein 1 Reactions: Not Available Gene Name: GLTPD1 Uniprot ID: Q5TA50 Protein Sequence: FASTA Gene Sequence: FASTA
Name: T-cell surface glycoprotein CD1c Reactions: Not Available Gene Name: CD1C Uniprot ID: P29017 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Lipopolysaccharide-binding protein Reactions: Not Available Gene Name: LBP Uniprot ID: P18428 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Putative uncharacterized protein DKFZp434L0435 Reactions: Not Available Gene Name: DKFZp434L0435 Uniprot ID: Q9UFH6 Protein Sequence: FASTA Gene Sequence: FASTA
Name: T-cell surface glycoprotein CD1b Reactions: Not Available Gene Name: CD1B Uniprot ID: P29016 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycolipid transfer protein Reactions: Not Available Gene Name: GLTP Uniprot ID: Q9NZD2 Protein Sequence: FASTA Gene Sequence: FASTA

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