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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:46:20 UTC
HMDB IDHMDB0004834
Secondary Accession Numbers
  • HMDB04834
Metabolite Identification
Common NameGalabiosylceramide (d18:1/18:0)
DescriptionGalabiosylceramide is a non-acidic diglycosphingolipids, i.e. a sphingolipid with two or more carbohydrate moieties attached to a ceramide unit. It is a vital component of cellular membranes of most eukaryotic organisms and some bacteria. Its abundance relative to other lipids is usually low other than in epithelial and neuronal cells. Galabiosylceramide has also been found in small amounts in kidney and pancreas, for example. Galabiosylceramide is one of the lipids that accumulates in excessive amounts in Fabry's disease. It is the precursor of the gala series of oligoglycosylceramides.An animal tissues, biosynthesis involves addition of a second monosaccharides unit from the appropriate sugar nucleotide to a monoglycosylceramide, catalysed by a glycosyl transferase, in the lumen of the Golgi apparatus. Glycolipids are important components of the body's immune defense system, either in haptenic reactivity or in antibody-producing potency, i.e. as cellular immunogens or antigens. Certain glycolipids are involved in the antigenicity of blood group determinants, while others bind to specific toxins or bacteria. Some also function as receptors for cellular recognition, and they can be specific for particular tissues or tumours.
Structure
Data?1563860780
Synonyms
ValueSource
N-[(4Z)-1-{[(2R,3R,4R,5R,6R)-3,4-dihydroxy-6-(hydroxymethyl)-5-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-3-hydroxyoctadec-4-en-2-yl]octadecanimidateGenerator
1-O-(4-O-alpha-D-Galactopyranosyl-beta-D-galactopyranosyl)-ceramideHMDB
1-O-(4-O-alpha-delta-Galactopyranosyl-beta-delta-galactopyranosyl)-ceramideHMDB
DigalactosylceramideHMDB
Gal-alpha1->4gal-beta1->1'cerHMDB
Chemical FormulaC48H91NO13
Average Molecular Weight890.235
Monoisotopic Molecular Weight889.649042003
IUPAC NameN-[(4Z)-1-{[(2R,3R,4R,5R,6R)-3,4-dihydroxy-6-(hydroxymethyl)-5-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-3-hydroxyoctadec-4-en-2-yl]octadecanamide
Traditional NameN-[(4Z)-1-{[(2R,3R,4R,5R,6R)-3,4-dihydroxy-6-(hydroxymethyl)-5-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-3-hydroxyoctadec-4-en-2-yl]octadecanamide
CAS Registry NumberNot Available
SMILES
CCCCCCCCCCCCCCCCCC(=O)NC(CO[C@@H]1O[C@H](CO)[C@H](O[C@H]2O[C@H](CO)[C@H](O)[C@H](O)[C@H]2O)[C@H](O)[C@H]1O)C(O)\C=C/CCCCCCCCCCCCC
InChI Identifier
InChI=1S/C48H91NO13/c1-3-5-7-9-11-13-15-17-18-20-22-24-26-28-30-32-40(53)49-36(37(52)31-29-27-25-23-21-19-16-14-12-10-8-6-4-2)35-59-47-45(58)43(56)46(39(34-51)61-47)62-48-44(57)42(55)41(54)38(33-50)60-48/h29,31,36-39,41-48,50-52,54-58H,3-28,30,32-35H2,1-2H3,(H,49,53)/b31-29-/t36?,37?,38-,39-,41+,42+,43-,44-,45-,46+,47-,48-/m1/s1
InChI KeyVOZHMDQUIRUFQW-NVIQELJCSA-N
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
ClassSphingolipids
Sub ClassGlycosphingolipids
Direct ParentGlycosyl-N-acylsphingosines
Alternative Parents
Substituents
  • Glycosyl-n-acylsphingosine
  • Fatty acyl glycoside
  • Fatty acyl glycoside of mono- or disaccharide
  • Alkyl glycoside
  • Disaccharide
  • Glycosyl compound
  • O-glycosyl compound
  • Fatty amide
  • N-acyl-amine
  • Oxane
  • Fatty acyl
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Carboxamide group
  • Organoheterocyclic compound
  • Polyol
  • Oxacycle
  • Carboxylic acid derivative
  • Acetal
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Alcohol
  • Organonitrogen compound
  • Carbonyl group
  • Organooxygen compound
  • Primary alcohol
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect

Organoleptic effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility0Not Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.0071 g/LALOGPS
logP6.26ALOGPS
logP8.21ChemAxon
logS-5.1ALOGPS
pKa (Strongest Acidic)11.92ChemAxon
pKa (Strongest Basic)-3ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count13ChemAxon
Hydrogen Donor Count9ChemAxon
Polar Surface Area227.86 ŲChemAxon
Rotatable Bond Count38ChemAxon
Refractivity239.8 m³·mol⁻¹ChemAxon
Polarizability106.75 ųChemAxon
Number of Rings2ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Lysosome
  • Golgi apparatus
Biospecimen LocationsNot Available
Tissue Locations
  • Brain
  • Heart
  • Kidney
Pathways
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB023435
KNApSAcK IDNot Available
Chemspider ID16744851
KEGG Compound IDC06126
BioCyc IDNot Available
BiGG ID2267787
Wikipedia LinkNot Available
METLIN ID7093
PubChem Compound20057275
PDB IDNot Available
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Ghislain J, Lingwood CA, Fish EN: Evidence for glycosphingolipid modification of the type 1 IFN receptor. J Immunol. 1994 Oct 15;153(8):3655-63. [PubMed:7930586 ]
  2. van der Bijl P, Lopes-Cardozo M, van Meer G: Sorting of newly synthesized galactosphingolipids to the two surface domains of epithelial cells. J Cell Biol. 1996 Mar;132(5):813-21. [PubMed:8603914 ]
  3. 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 ]
  4. 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 ]
  5. Lanne B, Jondal M, Karlsson KA: Gal alpha 4Gal-binding antibodies: specificity and use for the mapping of glycolipids of Burkitt lymphoma and other human tumors. Glycobiology. 1996 Jun;6(4):423-31. [PubMed:8842706 ]
  6. Falk P, Hoskins LC, Larson G: Enhancing effects of bile salts on the degradation of glycosphingolipids by glycosidases from bacteria of the human fecal flora. Biochim Biophys Acta. 1991 Jul 9;1084(2):139-48. [PubMed:1854798 ]
  7. Burger KN, van der Bijl P, van Meer G: Topology of sphingolipid galactosyltransferases in ER and Golgi: transbilayer movement of monohexosyl sphingolipids is required for higher glycosphingolipid biosynthesis. J Cell Biol. 1996 Apr;133(1):15-28. [PubMed:8601603 ]

Only showing the first 10 proteins. There are 61 proteins in total.

Enzymes

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:
GALC
Uniprot ID:
P54803
Molecular weight:
77062.86
General function:
Involved in galactosylceramide sulfotransferase activity
Specific function:
Catalyzes the sulfation of membrane glycolipids. Seems to prefer beta-glycosides at the non-reducing termini of sugar chains attached to a lipid moiety. Catalyzes the synthesis of galactosylceramide sulfate (sulfatide), a major lipid component of the myelin sheath and of monogalactosylalkylacylglycerol sulfate (seminolipid), present in spermatocytes (By similarity). Also acts on lactosylceramide, galactosyl 1-alkyl-2-sn-glycerol and galactosyl diacylglycerol (in vitro).
Gene Name:
GAL3ST1
Uniprot ID:
Q99999
Molecular weight:
48763.63
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:
GBGT1
Uniprot ID:
Q8N5D6
Molecular weight:
40126.9
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:
PIGL
Uniprot ID:
Q9Y2B2
Molecular weight:
28530.965
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
GLA
Uniprot ID:
P06280
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:
SMPD1
Uniprot ID:
P17405
Molecular weight:
69935.53
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:
UGCG
Uniprot ID:
Q16739
Molecular weight:
44853.255
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:
PIGQ
Uniprot ID:
Q9BRB3
Molecular weight:
65343.25
General function:
Involved in biosynthetic process
Specific function:
Necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis.
Gene Name:
PIGA
Uniprot ID:
P37287
Molecular weight:
54126.065
General function:
Involved in phosphatidylinositol N-acetylglucosaminyltr
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:
PIGH
Uniprot ID:
Q14442
Molecular weight:
21080.415

Only showing the first 10 proteins. There are 61 proteins in total.