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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:06:59 UTC
HMDB IDHMDB04958
Secondary Accession NumbersNone
Metabolite Identification
Common NameTetrahexosylceramide (d18:1/12:0)
DescriptionTetrahexosylceramide (d18:1/12:0) is a glycosphingolipid (ceramide and oligosaccharide)or oligoglycosylceramide with one or more sialic acids (i.e. n-acetylneuraminic acid) linked on the sugar chain. Tetrahexosylceramide contains a tetrasaccharide moiety bound in glycosidic linkage to the hydroxyl group of ceramide as the polar head group. It is a component the cell plasma membrane which modulates cell signal transduction events. Gangliosides have been found to be highly important in immunology. Ganglioside Gb4 carries a net-negative charge at pH 7.0 and is acidic. Gangliosides can amount to 6% of the weight of lipids from brain, but they are found at low levels in all animal tissues.Globosides are glycosphingolipids. There are four types of glycosphingolipids, the cerebrosides, sulfatides, globosides and gangliosides. Globosides are cerebrosides that contain additional carbohydrates, predominantly galactose, glucose or GalNAc. Lactosyl ceramide is a globoside found in erythrocyte plasma membranes. Globotriaosylceramide (also called ceramide trihexoside) contains glucose and two moles of galactose and accumulates, primarily in the kidneys, of patients suffering from Fabry disease.
Structure
Thumb
Synonyms
ValueSource
CQHHMDB
Cytolipin KHMDB
GalNAc-beta1->3gal-alpha1->3gal-beta1->4GLC-beta1->1'cerHMDB
Ganglioside GB4HMDB
Ganglioside GL 4HMDB
Gb4HMDB
GB4CerHMDB
globo-N-TetraosylceramideHMDB
GlobosidateHMDB
GlobosideHMDB
Globoside GB 4HMDB
Globoside GL 4HMDB
Globoside IHMDB
Globosidic acidHMDB
GlobotetraosylceramideHMDB
IGb4cerHMDB
N-Acetyl-D-galactosaminyl-1,3-D-galactosyl-1,4-D-galactosyl-1,4-D-glucosylceramideHMDB
N-Acetyl-delta-galactosaminyl-1,3-delta-galactosyl-1,4-delta-galactosyl-1,4-delta-glucosylceramideHMDB
N-Acetyl-galactosaminyl-1,3-D-galactosyl-1,4-D-galactosyl-1,4-D-glucosylceramideHMDB
N-Acetyl-galactosaminyl-1,3-delta-galactosyl-1,4-delta-galactosyl-1,4-delta-glucosylceramideHMDB
Parvovirus b19 receptorHMDB
Chemical FormulaC56H102N2O23
Average Molecular Weight1171.4087
Monoisotopic Molecular Weight1170.68733758
IUPAC NameN-[(2S,3R,4E)-1-{[(2R,4R,5S,6R)-5-{[(2S,3R,4R,5R,6R)-5-{[(2R,3R,4S,5S,6R)-4-{[(2S,3R,4R,5R,6R)-3-acetamido-4,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3-hydroxyoctadec-4-en-2-yl]dodecanamide
Traditional NameN-[(2S,3R,4E)-1-{[(2R,4R,5S,6R)-5-{[(2S,3R,4R,5R,6R)-5-{[(2R,3R,4S,5S,6R)-4-{[(2S,3R,4R,5R,6R)-3-acetamido-4,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-3-hydroxyoctadec-4-en-2-yl]dodecanamide
CAS Registry Number11034-93-8
SMILES
CCCCCCCCCCCCC\C=C\[C@@H](O)[C@H](CO[C@@H]1O[C@H](CO)[C@@H](O[C@@H]2O[C@H](CO)[C@H](O[C@H]3O[C@H](CO)[C@H](O)[C@H](O[C@@H]4O[C@H](CO)[C@H](O)[C@H](O)[C@H]4NC(C)=O)[C@H]3O)[C@H](O)[C@H]2O)[C@H](O)C1O)NC(=O)CCCCCCCCCCC
InChI Identifier
InChI=1S/C56H102N2O23/c1-4-6-8-10-12-14-15-16-17-19-20-22-24-26-35(64)34(58-40(65)27-25-23-21-18-13-11-9-7-5-2)32-74-54-47(71)45(69)50(38(30-61)77-54)79-55-48(72)46(70)51(39(31-62)78-55)80-56-49(73)52(43(67)37(29-60)76-56)81-53-41(57-33(3)63)44(68)42(66)36(28-59)75-53/h24,26,34-39,41-56,59-62,64,66-73H,4-23,25,27-32H2,1-3H3,(H,57,63)(H,58,65)/b26-24+/t34-,35+,36+,37+,38+,39+,41+,42-,43-,44+,45+,46+,47?,48+,49+,50+,51-,52-,53-,54+,55-,56+/m0/s1
InChI KeyInChIKey=MJVVJMMBLSKMDU-QVQHRZDJSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as glycosphingolipids. These are sphingolipids containing a saccharide moiety glycosidically attached to the sphingoid base. Although saccharide moieties are mostly O-glycosidically linked to the ceramide moiety, other sphingolipids with glycosidic bonds of other types (e.g. S-,C-, or N-type) has been reported.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassSphingolipids
Sub ClassGlycosphingolipids
Direct ParentGlycosphingolipids
Alternative Parents
Substituents
  • Simple glycosphingolipid
  • Glycosphingolipid
  • Oligosaccharide
  • Fatty acyl glycoside
  • N-acyl-alpha-hexosamine
  • Glucosamine
  • Alkyl glycoside
  • O-glycosyl compound
  • Glycosyl compound
  • Amino saccharide
  • Fatty acyl
  • Oxane
  • N-acyl-amine
  • Fatty amide
  • Acetamide
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Carboxamide group
  • 1,2-diol
  • Oxacycle
  • Organoheterocyclic compound
  • Carboxylic acid derivative
  • Carboxylic acid amide
  • Acetal
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Membrane
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityInsolubleNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.14 mg/mLALOGPS
logP2.23ALOGPS
logP1.72ChemAxon
logS-3.9ALOGPS
pKa (Strongest Acidic)11.59ChemAxon
pKa (Strongest Basic)-3.6ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count23ChemAxon
Hydrogen Donor Count15ChemAxon
Polar Surface Area395.03 Å2ChemAxon
Rotatable Bond Count39ChemAxon
Refractivity288.13 m3·mol-1ChemAxon
Polarizability129.74 Å3ChemAxon
Number of Rings4ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Membrane
Biofluid Locations
  • Blood
Tissue Location
  • Brain
  • Erythrocyte
  • Fibroblasts
  • Kidney
  • Liver
  • Nerve Cells
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.5 +/- 1.0 uMAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.5 (1.0-2.2) uMAdult (>18 years old)BothAbetalipoproteinemia details
BloodDetected and Quantified1.0 (1.0-1.1) uMAdult (>18 years old)BothHypobetalipoproteinemia details
Associated Disorders and Diseases
Disease References
Abetalipoproteinemia
  1. Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [178813 ]
Hypobetalipoproteinemia
  1. Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [178813 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB023545
KNApSAcK IDNot Available
Chemspider ID16744943
KEGG Compound IDC03272
BioCyc IDN-ACETYL-D-GALACTOSAMINYL-13-D-GALACT
BiGG IDNot Available
Wikipedia LinkCQH
NuGOwiki LinkHMDB04958
Metagene LinkHMDB04958
METLIN ID7211
PubChem Compound20057343
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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. [12829005 ]
  2. 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. [9106497 ]
  3. Choudhury A, Dominguez M, Puri V, Sharma DK, Narita K, Wheatley CL, Marks DL, Pagano RE: Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells. J Clin Invest. 2002 Jun;109(12):1541-50. [12070301 ]
  4. Ogawa-Goto K, Ohta Y, Kubota K, Funamoto N, Abe T, Taki T, Nagashima K: Glycosphingolipids of human peripheral nervous system myelins isolated from cauda equina. J Neurochem. 1993 Oct;61(4):1398-403. [7690848 ]
  5. Kannagi R, Fukuda MN, Hakomori S: A new glycolipid antigen isolated from human erythrocyte membranes reacting with antibodies directed to globo-N-tetraosylceramide (globoside). J Biol Chem. 1982 Apr 25;257(8):4438-42. [7068643 ]
  6. Kunishita T, Taketomi T: Sphingomyelin storage in a patient with myoclonus epilepsy as a main clinical symptom -- a varient in Niemann-Pick disease type C. Jpn J Exp Med. 1979 Apr;49(2):151-6. [113605 ]
  7. Zdebska E, Mendek-Czajkowska E, Ploski R, Woeniewicz B, Koscielak J: Heterozygosity of CDAN II (HEMPAS) gene may be detected by the analysis of erythrocyte membrane glycoconjugates from healthy carriers. Haematologica. 2002 Feb;87(2):126-30. [11836161 ]
  8. Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [178813 ]
  9. Hansson GC, Wazniowska K, Rock JA, Ness PM, Kickler TS, Shirey RS, Niebyl JR, Zopf D: The glycosphingolipid composition of the placenta of a blood group P fetus delivered by a blood group Pk1 woman and analysis of the anti-globoside antibodies found in maternal serum. Arch Biochem Biophys. 1988 Jan;260(1):168-76. [3341739 ]
  10. Tillack TW, Allietta M, Moran RE, Young WW Jr: Localization of globoside and Forssman glycolipids on erythrocyte membranes. Biochim Biophys Acta. 1983 Aug 24;733(1):15-24. [6603868 ]
  11. Marcus DM, Naiki M, Kundu SK: Abnormalities in the glycosphingolipid content of human Pk and p erythrocytes. Proc Natl Acad Sci U S A. 1976 Sep;73(9):3263-7. [1067617 ]
  12. Kundu SK, Steane SM, Bloom JE, Marcus DM: Abnormal glycolipid composition of erythrocytes with a weak P antigen. Vox Sang. 1978 Sep;35(3):160-7. [676244 ]
  13. Tatematsu M, Imaida K, Ito N, Togari H, Suzuki Y, Ogiu T: Sandhoff disease. Acta Pathol Jpn. 1981 May;31(3):503-12. [7270152 ]
  14. Zdebska E, Anselstetter V, Pacuszka T, Krauze R, Chelstowska A, Heimpel H, Koscielak J: Glycolipids and glycopeptides of red cell membranes in congenital dyserythropoietic anaemia type II (CDA II). Br J Haematol. 1987 Jul;66(3):385-91. [3620357 ]
  15. Snyder PD Jr, Krivit W, Sweeley CC: Generalized accumulation of neutral glycosphingolipids with G M2 ganglioside accumulation in the brain. J Lipid Res. 1972 Jan;13(1):128-36. [5059190 ]
  16. Leffler H, Lomberg H, Gotschlich E, Hagberg L, Jodal U, Korhonen T, Samuelsson BE, Schoolnik G, Svanborg-Eden C: Chemical and clinical studies on the interaction of Escherichia coli with host glycolipid receptors in urinary tract infection. Scand J Infect Dis Suppl. 1982;33:46-51. [6127802 ]
  17. Kijimoto-Ochiai S, Naiki M, Makita A: Defects of glycosyltransferase activities in human fibroblasts of Pk and p blood group phenotypes. Proc Natl Acad Sci U S A. 1977 Dec;74(12):5407-10. [271963 ]

Only showing the first 50 proteins. There are 63 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 catalytic activity
Specific function:
Removes terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides. Required for the breakdown of glycolipids.
Gene Name:
NAGA
Uniprot ID:
P17050
Molecular weight:
Not Available
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 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 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:
HEXB
Uniprot ID:
P07686
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:
HEXA
Uniprot ID:
P06865
Molecular weight:
Not Available
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
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:
PIGP
Uniprot ID:
P57054
Molecular weight:
18089.055
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:
PIGC
Uniprot ID:
Q92535
Molecular weight:
33582.18
General function:
Involved in galactosyltransferase activity
Specific function:
Transfers N-acetylgalactosamine onto globotriaosylceramide.
Gene Name:
B3GALNT1
Uniprot ID:
O75752
Molecular weight:
39511.55
General function:
Involved in lipid metabolic process
Specific function:
Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid.
Gene Name:
ASAH1
Uniprot ID:
Q13510
Molecular weight:
44045.27
General function:
Involved in catalytic activity
Specific function:
Converts sphingomyelin to ceramide. Also has phospholipase C activity toward palmitoyl lyso-phosphocholine. Does not appear to have nucleotide pyrophosphatase activity.
Gene Name:
ENPP7
Uniprot ID:
Q6UWV6
Molecular weight:
51493.415
General function:
Involved in metal ion binding
Specific function:
Converts sphingomyelin to ceramide. Hydrolyze 1-acyl-2-lyso-sn-glycero-3-phosphocholine (lyso-PC) and 1-O-alkyl-2-lyso-sn-glycero-3-phosphocholine (lyso-platelet-activating factor). The physiological substrate seems to be Lyso-PAF.
Gene Name:
SMPD2
Uniprot ID:
O60906
Molecular weight:
47645.29
General function:
Involved in protein binding
Specific function:
May mediate the intracellular trafficking of ceramide in a non-vesicular manner
Gene Name:
COL4A3BP
Uniprot ID:
Q9Y5P4
Molecular weight:
70834.4
General function:
Involved in sphingolipid activator protein activity
Specific function:
Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A. It extracts single GM2 molecules from membranes and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3
Gene Name:
GM2A
Uniprot ID:
P17900
Molecular weight:
20838.1
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
GBA
Uniprot ID:
P04062
Molecular weight:
59715.745
General function:
Involved in immune response
Specific function:
T-cell surface glycoprotein CD1e, soluble is required for the presentation of glycolipid antigens on the cell surface. The membrane-associated form is not active
Gene Name:
CD1E
Uniprot ID:
P15812
Molecular weight:
43626.1
General function:
Involved in cholesterol binding
Specific function:
May be involved in the regulation of the lipid composition of sperm membranes during the maturation in the epididymis
Gene Name:
NPC2
Uniprot ID:
P61916
Molecular weight:
16570.1
General function:
Involved in sphingosine N-acyltransferase activity
Specific function:
May be either a bona fide (dihydro)ceramide synthase or a modulator of its activity. When overexpressed in cells is involved in the production of sphingolipids containing mainly one fatty acid donor (N-linked stearoyl- (C18) ceramide) in a fumonisin B1-independent manner (By similarity).
Gene Name:
CERS1
Uniprot ID:
P27544
Molecular weight:
Not Available
General function:
Involved in immune response
Specific function:
Antigen-presenting protein that binds self and non-self glycolipids and presents them to T-cell receptors on natural killer T-cells
Gene Name:
CD1D
Uniprot ID:
P15813
Molecular weight:
37717.0
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
Mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the first alpha-1,4-mannose to GlcN-acyl-PI during GPI precursor assembly
Gene Name:
PIGM
Uniprot ID:
Q9H3S5
Molecular weight:
49459.2
General function:
Involved in transferase activity, transferring acyl groups
Specific function:
Probable acetyltransferase, which acetylates the inositol ring of phosphatidylinositol during biosynthesis of GPI-anchor. Acetylation during GPI-anchor biosynthesis is not essential for the subsequent mannosylation and is usually removed soon after the attachment of GPIs to proteins (By similarity).
Gene Name:
PIGW
Uniprot ID:
Q7Z7B1
Molecular weight:
Not Available
General function:
Involved in GPI anchor biosynthetic process
Specific function:
Essential component of glycosylphosphatidylinositol- mannosyltransferase 1 which transfers the first of the 4 mannoses in the GPI-anchor precursors during GPI-anchor biosynthesis. Probably acts by stabilizing the mannosyltransferase PIGM
Gene Name:
PIGX
Uniprot ID:
Q8TBF5
Molecular weight:
28788.1
General function:
Involved in transferase activity, transferring glycosyl groups
Specific function:
Mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers a fourth mannose to some trimannosyl-GPIs during GPI precursor assembly. The presence of a fourth mannose in GPI is facultative and only scarcely detected, suggesting that it only exists in some tissues
Gene Name:
PIGZ
Uniprot ID:
Q86VD9
Molecular weight:
63472.6
General function:
Involved in galactosyltransferase activity
Specific function:
Beta-1,3-N-acetylglucosaminyltransferase that plays a key role in the synthesis of lacto- or neolacto-series carbohydrate chains on glycolipids, notably by participating in biosynthesis of HNK-1 and Lewis X carbohydrate structures. Has strong activity toward lactosylceramide (LacCer) and neolactotetraosylceramide (nLc(4)Cer; paragloboside), resulting in the synthesis of Lc(3)Cer and neolactopentaosylceramide (nLc(5)Cer), respectively. Probably plays a central role in regulating neolacto-series glycolipid synthesis during embryonic development.
Gene Name:
B3GNT5
Uniprot ID:
Q9BYG0
Molecular weight:
44052.295
General function:
Involved in metal ion binding
Specific function:
Catalyzes the hydrolysis of sphingomyelin to form ceramide and phosphocholine. Ceramide mediates numerous cellular functions, such as apoptosis and growth arrest, and is capable of regulating these 2 cellular events independently. Also hydrolyzes sphingosylphosphocholine. Regulates the cell cycle by acting as a growth suppressor in confluent cells. Probably acts as a regulator of postnatal development and participates in bone and dentin mineralization.
Gene Name:
SMPD3
Uniprot ID:
Q9NY59
Molecular weight:
71080.1
General function:
Involved in galactosyltransferase activity
Specific function:
Catalyzes the transfer of Gal to GlcNAc-based acceptors with a preference for the core3 O-linked glycan GlcNAc(beta1,3)GalNAc structure. Can use glycolipid LC3Cer as an efficient acceptor
Gene Name:
B3GALT5
Uniprot ID:
Q9Y2C3
Molecular weight:
36188.9
General function:
Involved in transferase activity, transferring glycosyl groups
Specific function:
Mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the third alpha-1,2-mannose to Man2-GlcN-acyl-PI during GPI precursor assembly
Gene Name:
PIGB
Uniprot ID:
Q92521
Molecular weight:
65055.9
General function:
Involved in GPI anchor biosynthetic process
Specific function:
Involved in GPI-anchor biosynthesis through the transfer of ethanolamine phosphate to the third mannose of GPI
Gene Name:
PIGF
Uniprot ID:
Q07326
Molecular weight:
24889.3
General function:
Involved in catalytic activity
Specific function:
Ethanolamine phosphate transferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers ethanolamine phosphate to the GPI second mannose
Gene Name:
PIGG
Uniprot ID:
Q5H8A4
Molecular weight:
108171.7
General function:
Involved in catalytic activity
Specific function:
Ethanolamine phosphate transferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers ethanolamine phosphate to the first alpha-1,4-linked mannose of the glycosylphosphatidylinositol precursor of GPI-anchor
Gene Name:
PIGN
Uniprot ID:
O95427
Molecular weight:
105809.2
General function:
Involved in catalytic activity
Specific function:
Ethanolamine phosphate transferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers ethanolamine phosphate to the GPI third mannose which links the GPI-anchor to the C-terminus of the proteins by an amide bond
Gene Name:
PIGO
Uniprot ID:
Q8TEQ8
Molecular weight:
118697.6
General function:
Involved in protein binding
Specific function:
Component of the GPI transamidase complex. Essential for transfer of GPI to proteins, particularly for formation of carbonyl intermediates
Gene Name:
PIGS
Uniprot ID:
Q96S52
Molecular weight:
61655.5
General function:
Involved in protein binding
Specific function:
Component of the GPI transamidase complex. Essential for transfer of GPI to proteins, particularly for formation of carbonyl intermediates
Gene Name:
PIGT
Uniprot ID:
Q969N2
Molecular weight:
65699.0
General function:
Involved in GPI anchor biosynthetic process
Specific function:
Component of the GPI transamidase complex. May be involved in the recognition of either the GPI attachment signal or the lipid portion of GPI
Gene Name:
PIGU
Uniprot ID:
Q9H490
Molecular weight:
50051.2
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
Alpha-1,6-mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the second mannose to the glycosylphosphatidylinositol during GPI precursor assembly
Gene Name:
PIGV
Uniprot ID:
Q9NUD9
Molecular weight:
55712.1
General function:
Involved in GPI anchor biosynthetic process
Specific function:
Component of the GPI-GlcNAc transferase (GPI-GnT) complex in the endoplasmic reticulum, a complex that catalyzes transfer of GlcNAc from UDP-GlcNAc to an acceptor phosphatidylinositol, the first step in the production of GPI- anchors for cell surface proteins. May act by regulating the catalytic subunit PIGA
Gene Name:
PIGY
Uniprot ID:
Q3MUY2
Molecular weight:
8057.5
General function:
Involved in glycolipid transporter activity
Specific function:
Involved in TGN-to-plasma membrane transport and in the formation of post-Golgi constitutive carriers. May play a role in ensuring the coordination of the budding and the fission reactions
Gene Name:
PLEKHA8
Uniprot ID:
Q96JA3
Molecular weight:
58306.0
General function:
Involved in metal ion binding
Specific function:
Catalyzes the hydrolysis of membrane sphingomyelin to form phosphorylcholine and ceramide.
Gene Name:
SMPD4
Uniprot ID:
Q9NXE4
Molecular weight:
86192.575
General function:
Involved in ceramidase activity
Specific function:
May hydrolyze the sphingolipid ceramide into sphingosine and free fatty acid (By similarity).
Gene Name:
ASAH2C
Uniprot ID:
P0C7U2
Molecular weight:
Not Available
General function:
Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides
Specific function:
Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid. Unsaturated long-chain ceramides are the best substrates, saturated long-chain ceramides and unsaturated very long-chain ceramides are good substrates, whereas saturated very long-chain ceramides and short-chain ceramides were poor substrates. The substrate preference is D-erythro-C(18:1)-, C(20:1)-, C(20:4)-ceramide > D-erythro-C(16:0)-, C(18:0), C(20:0)-ceramide > D-erythro-C(24:1)-ceramide > D-erythro-C(12:0)-ceramide, D-erythro-C(14:0)-ceramides > D-erythro-C(24:0)-ceramide > D-erythro-C(6:0)-ceramide. Inhibits the maturation of protein glycosylation in the Golgi complex, including that of integrin beta-1 (ITGB1) and of LAMP1, by increasing the levels of sphingosine. Inhibits cell adhesion by reducing the level of ITGB1 in the cell surface. May have a role in cell proliferation and apoptosis that seems to depend on the balance between sphingosine and sphingosine-1-phosphate.
Gene Name:
ACER2
Uniprot ID:
Q5QJU3
Molecular weight:
31308.85
General function:
Involved in ceramidase activity
Specific function:
Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid at an optimal pH of 6.5-8.5. Acts as a key regulator of sphingolipid signaling metabolites by generating sphingosine at the cell surface. Acts as a repressor of apoptosis both by reducing C16-ceramide, thereby preventing ceramide-induced apoptosis, and generating sphingosine, a precursor of the antiapoptotic factor sphingosine 1-phosphate. Probably involved in the digestion of dietary sphingolipids in intestine by acting as a key enzyme for the catabolism of dietary sphingolipids and regulating the levels of bioactive sphingolipid metabolites in the intestinal tract.
Gene Name:
ASAH2
Uniprot ID:
Q9NR71
Molecular weight:
19024.55
General function:
Involved in catalytic activity
Specific function:
Non-lysosomal glucosylceramidase that catalyzes the conversion of glucosylceramide to free glucose and ceramide. Involved in sphingomyelin generation and prevention of glycolipid accumulation. May also catalyze the hydrolysis of bile acid 3-O-glucosides, however, the relevance of such activity is unclear in vivo.
Gene Name:
GBA2
Uniprot ID:
Q9HCG7
Molecular weight:
104648.13
General function:
Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides
Specific function:
Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid at an optimal pH of 8.0. Has a highly restricted substrate specificity for the natural stereoisomer of ceramide with D-erythro-sphingosine but not D-ribo-phytosphingosine or D-erythro-dihydrosphingosine as a backbone. May have a role in regulating the levels of bioactive lipids ceramide and sphingosine 1-phosphate, as well as complex sphingolipids (By similarity).
Gene Name:
ACER1
Uniprot ID:
Q8TDN7
Molecular weight:
31095.165
General function:
Involved in binding
Specific function:
Plays an inhibitory role on natural killer (NK) cells cytotoxicity. Activation results in specific acid sphingomyelinase/SMPD1 stimulation with subsequent marked elevation of intracellular ceramide. Activation also leads to AKT1/PKB and RPS6KA1/RSK1 kinases stimulation as well as markedly enhanced T-cell proliferation induced by anti-CD3. Acts as a lectin that binds to the terminal carbohydrate Gal-alpha(1,3)Gal epitope as well as to the N-acetyllactosamine epitope. Binds also to CLEC2D/LLT1 as a ligand and inhibits NK cell-mediated cytotoxicity as well as interferon-gamma secretion in target cells
Gene Name:
KLRB1
Uniprot ID:
Q12918
Molecular weight:
25414.6
General function:
Involved in sequence-specific DNA binding transcription factor activity
Specific function:
May be either a bona fide (dihydro)ceramide synthase or a modulator of its activity. When overexpressed in cells is involved in the production of sphingolipids containing mainly one fatty acid donor (N-linked palmitoyl- (C16) ceramide) in a fumonisin B1-independent manner (By similarity).
Gene Name:
CERS5
Uniprot ID:
Q8N5B7
Molecular weight:
Not Available

Only showing the first 50 proteins. There are 63 proteins in total.