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Record Information
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:43:33 UTC
Secondary Accession Numbers
  • HMDB04887
Metabolite Identification
Common NameTrihexosylceramide (d18:1/25:0)
DescriptionTrihexosylceramide 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 ).
Ceramide trihexosideHMDB
Fabry glycolipidHMDB
Ganglioside GL3HMDB
Shiga toxin receptorHMDB
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]pentacosanimidateGenerator, HMDB
Chemical FormulaC61H115NO18
Average Molecular Weight1150.5617
Monoisotopic Molecular Weight1149.811415881
IUPAC NameN-[(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 NameN-[(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 NumberNot Available
InChI Identifier
Chemical Taxonomy
Description Belongs to the class of organic compounds known as glycosyl-n-acylsphingosines. Glycosyl-N-acylsphingosines are compounds containing a sphingosine linked to a simple glucosyl moiety.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
Sub ClassGlycosphingolipids
Direct ParentGlycosyl-N-acylsphingosines
Alternative Parents
  • Glycosyl-n-acylsphingosine
  • Oligosaccharide
  • Fatty acyl glycoside
  • Alkyl glycoside
  • Glycosyl compound
  • O-glycosyl compound
  • Fatty amide
  • N-acyl-amine
  • Oxane
  • Fatty acyl
  • Secondary alcohol
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Oxacycle
  • Polyol
  • Organoheterocyclic compound
  • Carboxylic acid derivative
  • Acetal
  • Primary alcohol
  • Alcohol
  • Organopnictogen compound
  • Organic oxide
  • Organic oxygen compound
  • Hydrocarbon derivative
  • Organonitrogen compound
  • Carbonyl group
  • Organooxygen compound
  • Organic nitrogen compound
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available

Biological location


Route of exposure

Physical Properties
Experimental Molecular Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility0Not Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Water Solubility0.0096 g/LALOGPS
logP10(6.02) g/LALOGPS
logP10(9.55) g/LChemAxon
logS10(-5.1) g/LALOGPS
pKa (Strongest Acidic)11.76ChemAxon
pKa (Strongest Basic)-3.6ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count18ChemAxon
Hydrogen Donor Count12ChemAxon
Polar Surface Area307.01 ŲChemAxon
Rotatable Bond Count48ChemAxon
Refractivity304.42 m³·mol⁻¹ChemAxon
Polarizability138.14 ųChemAxon
Number of Rings3ChemAxon
Rule of FiveNoChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleYesChemAxon
Predicted Chromatographic Properties

Predicted Kovats Retention Indices

Not Available

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Trihexosylceramide (d18:1/25:0) 10V, Positive-QTOFsplash10-0gyk-2901002011-546a4e9f7b31f9e891472021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Trihexosylceramide (d18:1/25:0) 20V, Positive-QTOFsplash10-071j-1901001011-8cd741d680c7f69526e02021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Trihexosylceramide (d18:1/25:0) 40V, Positive-QTOFsplash10-01qd-5910001000-b50a971c1f7dde6b9e962021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Trihexosylceramide (d18:1/25:0) 10V, Negative-QTOFsplash10-0002-0900000002-3e1db00f58e9e3f908592021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Trihexosylceramide (d18:1/25:0) 20V, Negative-QTOFsplash10-0002-4901000010-83fab776a4f77bbfb0882021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Trihexosylceramide (d18:1/25:0) 40V, Negative-QTOFsplash10-08pr-5943144152-b34589b043bc0072a3602021-09-25Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biospecimen Locations
  • Urine
Tissue Locations
  • Brain
  • Epidermis
  • Kidney
  • Liver
  • Neuron
Normal Concentrations
UrineDetected and Quantified0.0001842-0.0003947 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDNot Available
KNApSAcK IDNot Available
Chemspider ID16744899
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound20057320
PDB IDNot Available
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
Good Scents IDNot Available
Synthesis ReferenceNot Available
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. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. 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 ]
  11. 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 ]
  12. 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 ]
  13. 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 ]
  14. 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 ]

Only showing the first 10 proteins. There are 64 proteins in total.


General function:
Involved in galactosylceramidase activity
Specific function:
Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
Catalyzes the formation of some glycolipid via the addition of N-acetylgalactosamine (GalNAc) in alpha-1,3-linkage to some substrate. Glycolipids probably serve for adherence of some pathogens
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in N-acetylglucosaminylphosphatidylinositol de
Specific function:
Involved in the second step of GPI biosynthesis. De-N-acetylation of N-acetylglucosaminyl-phosphatidylinositol.
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
Uniprot ID:
Molecular weight:
Not Available
General function:
Involved in hydrolase activity
Specific function:
Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Cell wall/membrane/envelope biogenesis
Specific function:
Catalyzes the first glycosylation step in glycosphingolipid biosynthesis, the transfer of glucose to ceramide. May also serve as a "flippase".
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in beta-N-acetylhexosaminidase activity
Specific function:
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.
Gene Name:
Uniprot ID:
Molecular weight:
Not Available
General function:
Involved in beta-N-acetylhexosaminidase activity
Specific function:
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.
Gene Name:
Uniprot ID:
Molecular weight:
Not Available
General function:
Involved in galactosyltransferase activity
Specific function:
Necessary for the biosynthesis of the Pk antigen of blood histogroup P. Catalyzes the transfer of galactose to lactosylceramide and galactosylceramide. Necessary for the synthesis of the receptor for bacterial verotoxins.
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltransferase activity
Specific function:
Part of the complex catalyzing the transfer of N-acetylglucosamine from UDP-N-acetylglucosamine to phosphatidylinositol, the first step of GPI biosynthesis.
Gene Name:
Uniprot ID:
Molecular weight:

Only showing the first 10 proteins. There are 64 proteins in total.