Record Information
Version3.6
Creation Date2008-09-26 14:11:48 UTC
Update Date2013-05-21 22:46:33 UTC
HMDB IDHMDB10712
Secondary Accession NumbersNone
Metabolite Identification
Common NameGalactosylceramide (d18:1/24:1(15Z))
DescriptionGalactosylceramides (GalCer) are non-acidic monoglycosphingolipids, i.e. a sphingolipid with one carbohydrate moiety attached to a ceramide unit. They are an intermediate in sphingolipid metabolism and is the second to last step in the synthesis of digalactosylceramidesulfate. GalCer is generated from ceramide via the enzyme UDP-galactose ceramide galactosyltransferase [EC:2.4.1.47]. It can be converted to digalactosylceramide via the enzyme glycosyltransferases [EC 2.4.1.-]. Galactosylceramide is the principal glycosphingolipid in brain tissue, hence the trivial name "cerebroside", which was first conferred on it in 1874. Galactosylceramides are found in all nervous tissues, but they can amount to 2% of the dry weight of grey matter and 12% of white matter. They are major constituents of oligodendrocytes. Synthesis of galactosylceramide takes place on the lumenal surface of the endoplasmic reticulum, although it has free access to the cytosolic surface by an energy-independent flip-flop process. GalCer sits in the extracellular leaflet of cell membranes in nanometer sized domains or rafts. The local clustering of GalCer within rafts is thought to facilitate the initial adhesion of certain viruses, including HIV-1 and bacteria to cells through multivalent interactions between receptor proteins and GalCer. A defect in the degradation of cerbrosides leads to a disorder called Krabbe disease. Krabbe disease (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare, often fatal degenerative disorder that affects the myelin sheath of the nervous system. Krabbe disease is caused by mutations in the GALC gene, which causes a deficiency of galactosylceramidase. Infants with Krabbe disease are normal at birth. Symptoms begin between the ages of 3 and 6 months with irritability, fevers, limb stiffness, seizures, feeding difficulties, vomiting, and slowing of mental and motor development. There are also juvenile- and adult-onset cases of Krabbe disease, which have similar symptoms but slower progression. In infants, the disease is generally fatal before age 2. Patients with late-onset Krabbe disease tend to have a slower progression of the disease and live significantly longer.Cerebrosides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Cerebrosides have a single sugar group linked to ceramide. The most common are galactocerebrosides (containing galactose), the least common are glucocerebrosides (containing glucose). Galactocerebrosides are found predominantly in neuronal cell membranes. In contrast glucocerebrosides are not normally found in membranes. Instead, they are typically intermediates in the synthesis or degradation of more complex glycosphingolipids. Galactocerebrosides are synthesized from ceramide and UDP-galactose. Excess lysosomal accumulation of glucocerebrosides is found in Gaucher disease.
Structure
Thumb
Synonyms
  1. a-GalCer
  2. alpha-GalCer
  3. Cerebroside
  4. D-Galactosyl-N-acylsphingosine
  5. D-Galactosylceramide
  6. delta-Galactosyl-N-acylsphingosine
  7. delta-Galactosylceramide
  8. Gal-b-Cer
  9. Gal-beta-1-1'Cer
  10. Gal-beta-Cer
  11. Galactocerebroside
  12. Galactosylceramide
  13. GalCer
  14. N-(15Z-Tetracosenoyl)-1-b-galactosyl-sphing-4-enine
  15. N-(15Z-Tetracosenoyl)-1-beta-galactosyl-sphing-4-enine
Chemical FormulaC48H91NO8
Average Molecular Weight810.238
Monoisotopic Molecular Weight809.674468893
IUPAC Name(15Z)-N-[(4Z)-3-hydroxy-1-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl]tetracos-15-enamide
Traditional IUPAC Name(15Z)-N-[(4Z)-3-hydroxy-1-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl]tetracos-15-enamide
CAS Registry NumberNot Available
SMILES
CCCCCCCCCCCCC\C=C/C(O)C(CO[C@@H]1O[C@H](CO)[C@H](O)[C@H](O)[C@H]1O)NC(=O)CCCCCCCCCCCCC\C=C/CCCCCCCC
InChI Identifier
InChI=1S/C48H91NO8/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-23-24-26-28-30-32-34-36-38-44(52)49-41(40-56-48-47(55)46(54)45(53)43(39-50)57-48)42(51)37-35-33-31-29-27-25-16-14-12-10-8-6-4-2/h17-18,35,37,41-43,45-48,50-51,53-55H,3-16,19-34,36,38-40H2,1-2H3,(H,49,52)/b18-17-,37-35-/t41?,42?,43-,45+,46+,47-,48-/m1/s1
InChI KeyWBOZIXHPUPAOIA-DFJDAMKWSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassSphingolipids
Sub ClassNeutral Glycosphingolipids
Other Descriptors
  • Aliphatic Heteromonocyclic Compounds
Substituents
  • 1,2 Diol
  • Acetal
  • Alkyl Glycoside
  • Allyl Alcohol
  • Carboxamide Group
  • Ceramide
  • Fatty Acyl Glycoside
  • Glycosyl Compound
  • Hexose Monosaccharide
  • N Acyl Amine
  • O Glycosyl Compound
  • Oxane
  • Primary Alcohol
  • Saccharide
  • Secondary Alcohol
  • Secondary Carboxylic Acid Amide
Direct ParentSimple Glycosylceramides
Ontology
StatusExpected and Not Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Energy source
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane component
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
water solubility2.400E-04 g/LALOGPS
logP9.12ALOGPS
logP12.29ChemAxon
logS-6.5ALOGPS
pKa (strongest acidic)12.18ChemAxon
pKa (strongest basic)0.019ChemAxon
physiological charge0ChemAxon
hydrogen acceptor count8ChemAxon
hydrogen donor count6ChemAxon
polar surface area148.71ChemAxon
rotatable bond count40ChemAxon
refractivity236.11ChemAxon
polarizability103.52ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid LocationsNot Available
Tissue Location
  • All Tissues
PathwaysNot Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB027862
KNApSAcK IDNot Available
Chemspider ID24765748
KEGG Compound IDC02686
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB10712
Metagene LinkHMDB10712
METLIN IDNot Available
PubChem Compound53480656
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Wang J, Guillaume J, Pauwels N, Van Calenbergh S, Van Rhijn I, Zajonc DM: Crystal structures of bovine CD1d reveal altered alphaGalCer presentation and a restricted A' pocket unable to bind long-chain glycolipids. PLoS One. 2012;7(10):e47989. doi: 10.1371/journal.pone.0047989. Epub 2012 Oct 23. Pubmed: 23110152
  2. Okamoto Y, Fujikawa A, Kurosaki M, Yamasaki K, Sakurai D, Horiguchi S, Nakayama T: Nasal submucosal administration of antigen-presenting cells induces effective immunological responses in cancer immunotherapy. Adv Otorhinolaryngol. 2011;72:149-52. doi: 10.1159/000324775. Epub 2011 Aug 18. Pubmed: 21865716
  3. Lopez-Sagaseta J, Kung JE, Savage PB, Gumperz J, Adams EJ: The molecular basis for recognition of CD1d/alpha-galactosylceramide by a human non-Valpha24 T cell receptor. PLoS Biol. 2012;10(10):e1001412. doi: 10.1371/journal.pbio.1001412. Epub 2012 Oct 23. Pubmed: 23109910
  4. Tonti E, Fedeli M, Napolitano A, Iannacone M, von Andrian UH, Guidotti LG, Abrignani S, Casorati G, Dellabona P: Follicular helper NKT cells induce limited B cell responses and germinal center formation in the absence of CD4(+) T cell help. J Immunol. 2012 Apr 1;188(7):3217-22. doi: 10.4049/jimmunol.1103501. Epub 2012 Feb 29. Pubmed: 22379027
  5. Freigang S, Landais E, Zadorozhny V, Kain L, Yoshida K, Liu Y, Deng S, Palinski W, Savage PB, Bendelac A, Teyton L: Scavenger receptors target glycolipids for natural killer T cell activation. J Clin Invest. 2012 Nov 1;122(11):3943-54. doi: 10.1172/JCI62267. Epub 2012 Oct 15. Pubmed: 23064364
  6. Yang JQ, Kim PJ, Singh RR: Brief treatment with iNKT cell ligand alpha-galactosylceramide confers a long-term protection against lupus. J Clin Immunol. 2012 Feb;32(1):106-13. doi: 10.1007/s10875-011-9590-y. Epub 2011 Oct 15. Pubmed: 22002593
  7. Patel O, Pellicci DG, Uldrich AP, Sullivan LC, Bhati M, McKnight M, Richardson SK, Howell AR, Mallevaey T, Zhang J, Bedel R, Besra GS, Brooks AG, Kjer-Nielsen L, McCluskey J, Porcelli SA, Gapin L, Rossjohn J, Godfrey DI: Vbeta2 natural killer T cell antigen receptor-mediated recognition of CD1d-glycolipid antigen. Proc Natl Acad Sci U S A. 2011 Nov 22;108(47):19007-12. doi: 10.1073/pnas.1109066108. Epub 2011 Nov 7. Pubmed: 22065767
  8. Detre C, Keszei M, Garrido-Mesa N, Kis-Toth K, Castro W, Agyemang AF, Veerapen N, Besra GS, Carroll MC, Tsokos GC, Wang N, Leadbetter EA, Terhorst C: SAP expression in invariant NKT cells is required for cognate help to support B-cell responses. Blood. 2012 Jul 5;120(1):122-9. doi: 10.1182/blood-2011-11-395913. Epub 2012 May 21. Pubmed: 22613797
  9. Tyznik AJ, Farber E, Girardi E, Birkholz A, Li Y, Chitale S, So R, Arora P, Khurana A, Wang J, Porcelli SA, Zajonc DM, Kronenberg M, Howell AR: Glycolipids that elicit IFN-gamma-biased responses from natural killer T cells. Chem Biol. 2011 Dec 23;18(12):1620-30. doi: 10.1016/j.chembiol.2011.10.015. Pubmed: 22195564
  10. Nagy L: Would eating carrots protect your liver? A new role involving NKT cells for retinoic acid in hepatitis. Eur J Immunol. 2012 Jul;42(7):1677-80. doi: 10.1002/eji.201242705. Pubmed: 22806070
  11. Choi DH, Kim KS, Yang SH, Chung DH, Song B, Sprent J, Cho JH, Sung YC: Dendritic cell internalization of alpha-galactosylceramide from CD8 T cells induces potent antitumor CD8 T-cell responses. Cancer Res. 2011 Dec 15;71(24):7442-51. doi: 10.1158/0008-5472.CAN-11-1459. Epub 2011 Oct 25. Pubmed: 22028323
  12. Yu ED, Girardi E, Wang J, Mac TT, Yu KO, Van Calenbergh S, Porcelli SA, Zajonc DM: Structural basis for the recognition of C20:2-alphaGalCer by the invariant natural killer T cell receptor-like antibody L363. J Biol Chem. 2012 Jan 6;287(2):1269-78. doi: 10.1074/jbc.M111.308783. Epub 2011 Nov 22. Pubmed: 22110136
  13. Kim YJ, Han SH, Kang HW, Lee JM, Kim YS, Seo JH, Seong YK, Ko HJ, Choi TH, Moon C, Kang CY: NKT ligand-loaded, antigen-expressing B cells function as long-lasting antigen presenting cells in vivo. Cell Immunol. 2011;270(2):135-44. doi: 10.1016/j.cellimm.2011.04.006. Epub 2011 Apr 22. Pubmed: 21741036
  14. Chen Q, Ross AC: All-trans-retinoic acid and the glycolipid alpha-galactosylceramide combined reduce breast tumor growth and lung metastasis in a 4T1 murine breast tumor model. Nutr Cancer. 2012;64(8):1219-27. doi: 10.1080/01635581.2012.718404. Pubmed: 23163850

Only showing the first 50 proteins. There are 70 proteins in total.

Enzymes

Gene Name:
GALC
Uniprot ID:
P54803
Gene Name:
ARSD
Uniprot ID:
P51689
Gene Name:
NEU2
Uniprot ID:
Q9Y3R4
Gene Name:
GAL3ST1
Uniprot ID:
Q99999
Gene Name:
ARSA
Uniprot ID:
P15289
Gene Name:
NEU4
Uniprot ID:
Q8WWR8
Gene Name:
ARSE
Uniprot ID:
P51690
Gene Name:
GLA
Uniprot ID:
P06280
Gene Name:
SMPD1
Uniprot ID:
P17405
Gene Name:
UGCG
Uniprot ID:
Q16739
Gene Name:
ASAH1
Uniprot ID:
Q13510
Gene Name:
SMPD2
Uniprot ID:
O60906
Gene Name:
COL4A3BP
Uniprot ID:
Q9Y5P4
Gene Name:
GM2A
Uniprot ID:
P17900
Gene Name:
GBA
Uniprot ID:
P04062
Gene Name:
NPC2
Uniprot ID:
P61916
Gene Name:
CERS1
Uniprot ID:
P27544
Gene Name:
CD1D
Uniprot ID:
P15813
Gene Name:
PIGM
Uniprot ID:
Q9H3S5
Gene Name:
PIGZ
Uniprot ID:
Q86VD9
Gene Name:
SMPD3
Uniprot ID:
Q9NY59
Gene Name:
B3GALT5
Uniprot ID:
Q9Y2C3
Gene Name:
B4GALT6
Uniprot ID:
Q9UBX8
Gene Name:
PIGB
Uniprot ID:
Q92521
Gene Name:
PIGS
Uniprot ID:
Q96S52
Gene Name:
PIGT
Uniprot ID:
Q969N2
Gene Name:
PIGV
Uniprot ID:
Q9NUD9
Gene Name:
SMPD4
Uniprot ID:
Q9NXE4
Gene Name:
ASAH2C
Uniprot ID:
P0C7U2

Only showing the first 50 proteins. There are 70 proteins in total.