Human Metabolome Database: Showing metabocard for Galactosylceramide (d18:1/26:1(17Z)) (HMDB0010713)

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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (70) Show 70 proteins XML

enzymes (70) Show 70 proteins

Record Information

Version

4.0

Status

Expected but not Quantified

Creation Date

2008-09-26 14:11:49 UTC

Update Date

2020-02-26 21:34:24 UTC

HMDB ID

HMDB0010713

Secondary Accession Numbers

HMDB10713

Metabolite Identification

Common Name

Galactosylceramide (d18:1/26:1(17Z))

Description

Galactosylceramides (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

MOL SDF PDB SMILES InChI

Synonyms

Value	Source
a-GalCer	HMDB
alpha-GalCer	HMDB
Cerebroside	HMDB
D-Galactosyl-N-acylsphingosine	HMDB
D-Galactosylceramide	HMDB
delta-Galactosyl-N-acylsphingosine	HMDB
delta-Galactosylceramide	HMDB
Gal-b-cer	HMDB
Gal-beta-1-1'cer	HMDB
Gal-beta-cer	HMDB
Galactocerebroside	HMDB
Galactosylceramide	HMDB
GalCer	HMDB
N-(17Z-Hexacosenoyl)-1-b-galactosyl-sphing-4-enine	HMDB
N-(17Z-Hexacosenoyl)-1-beta-galactosyl-sphing-4-enine	HMDB
(17Z)-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]hexacos-17-enimidate	Generator

Chemical Formula

C₅₀H₉₅NO₈

Average Molecular Weight

838.2912

Monoisotopic Molecular Weight

837.705769021

IUPAC Name

(17Z)-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]hexacos-17-enamide

Traditional Name

(17Z)-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]hexacos-17-enamide

CAS Registry Number

Not 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)CCCCCCCCCCCCCCC\C=C/CCCCCCCC

InChI Identifier

InChI=1S/C50H95NO8/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-23-24-25-26-28-30-32-34-36-38-40-46(54)51-43(42-58-50-49(57)48(56)47(55)45(41-52)59-50)44(53)39-37-35-33-31-29-27-16-14-12-10-8-6-4-2/h17-18,37,39,43-45,47-50,52-53,55-57H,3-16,19-36,38,40-42H2,1-2H3,(H,51,54)/b18-17-,39-37-/t43?,44?,45-,47+,48+,49-,50-/m1/s1

InChI Key

LVROXDRMMJVBGH-WRDHKATFSA-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.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Sphingolipids

Sub Class

Glycosphingolipids

Direct Parent

Glycosyl-N-acylsphingosines

Alternative Parents

Substituents

Glycosyl-n-acylsphingosine
Fatty acyl glycoside
Fatty acyl glycoside of mono- or disaccharide
Alkyl glycoside
Hexose monosaccharide
Glycosyl compound
O-glycosyl compound
Fatty amide
Fatty acyl
Monosaccharide
N-acyl-amine
Oxane
Carboxamide group
Secondary carboxylic acid amide
Secondary alcohol
Acetal
Carboxylic acid derivative
Oxacycle
Organoheterocyclic compound
Polyol
Hydrocarbon derivative
Organic oxide
Organopnictogen compound
Alcohol
Organic oxygen compound
Organic nitrogen compound
Primary alcohol
Carbonyl group
Organooxygen compound
Organonitrogen compound
Aliphatic heteromonocyclic compound

Molecular Framework

Aliphatic heteromonocyclic compounds

External Descriptors

Not Available

Ontology

Physiological effect

Organoleptic effect:

Touch:

Smooth

Disposition

Route of exposure:

Enteral:

Ingestion

Source:

Biological:

Animal

Biological location:

Tissue and substructures:

All tissues

Cell and elements:

Extracellular

Subcellular:

Process

Naturally occurring process:

Biological process:

Chemical reaction:

Lipid peroxidation

Biochemical pathway:

Lipid metabolism pathway

Biochemical process:

Cellular process:

Cell signaling

Role

Industrial application:

Food and nutrition:

Biological role:

Molecular messenger:

Signaling molecule

Physical Properties

State

Solid

Experimental Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Predicted Properties

Property	Value	Source
Water Solubility	0.00017 g/L	ALOGPS
logP	9.34	ALOGPS
logP	13.18	ChemAxon
logS	-6.7	ALOGPS
pKa (Strongest Acidic)	12.18	ChemAxon
pKa (Strongest Basic)	0.019	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	8	ChemAxon
Hydrogen Donor Count	6	ChemAxon
Polar Surface Area	148.71 Å²	ChemAxon
Rotatable Bond Count	42	ChemAxon
Refractivity	245.32 m³·mol⁻¹	ChemAxon
Polarizability	107.8 Å³	ChemAxon
Number of Rings	1	ChemAxon
Bioavailability	No	ChemAxon
Rule of Five	No	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Not Available

Biological Properties

Cellular Locations

Extracellular
Membrane

Biospecimen Locations

Not Available

Tissue Locations

All Tissues

Pathways

Not Available

Name	SMPDB/Pathwhiz	KEGG

Normal Concentrations

Not Available

Abnormal Concentrations

Not Available

Associated Disorders and Diseases

Disease References

None

Associated OMIM IDs

None

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB027863

KNApSAcK ID

Not Available

Chemspider ID

24765749

KEGG Compound ID

C02686

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

Not Available

PubChem Compound

53480657

PDB ID

Not Available

ChEBI ID

Not Available

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

References

Synthesis Reference

Not Available

Material Safety Data Sheet (MSDS)

Not Available

General References

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 ]
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 ]
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 ]
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 ]
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;122(11):3943-54. doi: 10.1172/JCI62267. Epub 2012 Oct 15. [PubMed:23064364 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
Divecha N, Irvine RF: Phospholipid signaling. Cell. 1995 Jan 27;80(2):269-78. [PubMed:7834746 ]
Ghosh S, Strum JC, Bell RM: Lipid biochemistry: functions of glycerolipids and sphingolipids in cellular signaling. FASEB J. 1997 Jan;11(1):45-50. [PubMed:9034165 ]
Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
Watson AD: Thematic review series: systems biology approaches to metabolic and cardiovascular disorders. Lipidomics: a global approach to lipid analysis in biological systems. J Lipid Res. 2006 Oct;47(10):2101-11. Epub 2006 Aug 10. [PubMed:16902246 ]
Sethi JK, Vidal-Puig AJ: Thematic review series: adipocyte biology. Adipose tissue function and plasticity orchestrate nutritional adaptation. J Lipid Res. 2007 Jun;48(6):1253-62. Epub 2007 Mar 20. [PubMed:17374880 ]
Lingwood D, Simons K: Lipid rafts as a membrane-organizing principle. Science. 2010 Jan 1;327(5961):46-50. doi: 10.1126/science.1174621. [PubMed:20044567 ]
Cevc, Gregor (1993). Phospholipids Handbook. Marcel Dekker.
Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.

Only showing the first 10 proteins. There are 70 proteins in total.

Show all enzymes and transporters

Enzymes

Enzyme Details

1. Galactocerebrosidase

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

Enzyme Details

2. Arylsulfatase D

General function:: Involved in catalytic activity
Specific function:: Not Available
Gene Name:: ARSD
Uniprot ID:: P51689
Molecular weight:: 64859.3

Enzyme Details

3. Sialidase-2

General function:: Involved in exo-alpha-sialidase activity
Specific function:: Hydrolyzes sialylated compounds.
Gene Name:: NEU2
Uniprot ID:: Q9Y3R4
Molecular weight:: Not Available

Enzyme Details

4. Galactosylceramide sulfotransferase

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

Enzyme Details

5. Arylsulfatase A

General function:: Involved in catalytic activity
Specific function:: Hydrolyzes cerebroside sulfate.
Gene Name:: ARSA
Uniprot ID:: P15289
Molecular weight:: 53805.87

Enzyme Details

6. Sialidase-4

General function:: Involved in exo-alpha-sialidase activity
Specific function:: May function in lysosomal catabolism of sialylated glycoconjugates. Has sialidase activity towards synthetic substrates, such as 2'-(4-methylumbelliferyl)-alpha-D-N-acetylneuraminic acid (4-MU-NANA or 4MU-NeuAc). Has a broad substrate specificity being active on glycoproteins, oligosaccharides and sialylated glycolipids.
Gene Name:: NEU4
Uniprot ID:: Q8WWR8
Molecular weight:: Not Available

Enzyme Details

7. Arylsulfatase E

General function:: Involved in catalytic activity
Specific function:: May be essential for the correct composition of cartilage and bone matrix during development. Has no activity toward steroid sulfates
Gene Name:: ARSE
Uniprot ID:: P51690
Molecular weight:: 65668.4

Enzyme Details

8. Globoside alpha-1,3-N-acetylgalactosaminyltransferase 1

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

Enzyme Details

9. N-acetylglucosaminyl-phosphatidylinositol de-N-acetylase

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

Enzyme Details

10. Alpha-galactosidase A

General function:: Involved in catalytic activity
Specific function:: Not Available
Gene Name:: GLA
Uniprot ID:: P06280
Molecular weight:: Not Available

Only showing the first 10 proteins. There are 70 proteins in total.

Show all enzymes and transporters

Showing metabocard for Galactosylceramide (d18:1/26:1(17Z)) (HMDB0010713)

Structure for HMDB0010713 (Galactosylceramide (d18:1/26:1(17Z)))

Enzymes