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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 IDHMDB04969
Secondary Accession NumbersNone
Metabolite Identification
Common NameGlucosylceramide (d18:1/12:0)
DescriptionGlucosylceramide (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. 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 GL1a 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.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
ValueSource
1-O-beta-D-Glucopyranosyl-N-(dodecanoyl)sphingosineChEBI
beta-GlcCer (C12)ChEBI
beta-GlcCer (C12:0)ChEBI
C12 beta-GlcCerChEBI
C12-beta-Glucosyl ceramideChEBI
1-O-b-D-Glucopyranosyl-N-(dodecanoyl)sphingosineGenerator
1-O-β-D-glucopyranosyl-N-(dodecanoyl)sphingosineGenerator
1-O-b-D-Glucopyranosyl-ceramideHMDB
1-O-beta-delta-Glucopyranosyl-ceramideHMDB
Ganglioside GL1aHMDB
Gaucher cerebrosideHMDB
GLC-beta1->1'cerHMDB
GlcCeramideHMDB
GlucocerebrosideHMDB
GlucosylceramideHMDB
Chemical FormulaC36H69NO8
Average Molecular Weight643.935
Monoisotopic Molecular Weight643.502318189
IUPAC NameN-[(2S,3R,4E)-3-hydroxy-1-{[(2R,3R,4S,5S,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl]dodecanamide
Traditional NameN-[(2S,3R,4E)-3-hydroxy-1-{[(2R,3R,4S,5S,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl]dodecanamide
CAS Registry Number85305-87-9
SMILES
CCCCCCCCCCCCC\C=C\[C@@H](O)[C@H](CO[C@@H]1O[C@H](CO)[C@@H](O)[C@H](O)[C@H]1O)NC(=O)CCCCCCCCCCC
InChI Identifier
InChI=1S/C36H69NO8/c1-3-5-7-9-11-13-14-15-16-18-19-21-23-25-30(39)29(28-44-36-35(43)34(42)33(41)31(27-38)45-36)37-32(40)26-24-22-20-17-12-10-8-6-4-2/h23,25,29-31,33-36,38-39,41-43H,3-22,24,26-28H2,1-2H3,(H,37,40)/b25-23+/t29-,30+,31+,33+,34-,35+,36+/m0/s1
InChI KeyInChIKey=IYCYEZLMOLRFAN-IUGLYQEMSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as simple glycosylceramides. These are compounds containing a ceramide linked to a simple glycosyl moiety.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassSphingolipids
Sub ClassGlycosphingolipids
Direct ParentSimple glycosylceramides
Alternative Parents
Substituents
  • Simple glycosylceramide
  • Fatty acyl glycoside of mono- or disaccharide
  • Fatty acyl glycoside
  • Alkyl glycoside
  • O-glycosyl compound
  • Glycosyl compound
  • Fatty acyl
  • Oxane
  • N-acyl-amine
  • Monosaccharide
  • Fatty amide
  • Saccharide
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Polyol
  • 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 Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • 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 SolubilityInsolubleNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.0034 mg/mLALOGPS
logP6.44ALOGPS
logP7.32ChemAxon
logS-5.3ALOGPS
pKa (Strongest Acidic)12.18ChemAxon
pKa (Strongest Basic)0.019ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count8ChemAxon
Hydrogen Donor Count6ChemAxon
Polar Surface Area148.71 Å2ChemAxon
Rotatable Bond Count29ChemAxon
Refractivity179.79 m3·mol-1ChemAxon
Polarizability79.8 Å3ChemAxon
Number of Rings1ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
Tissue Location
  • Bone Marrow
  • Brain
  • Liver
  • Nerve Cells
  • Spleen
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3.6 (2.5-7.2) uMAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified4.3 (2.9-5.0) uMAdult (>18 years old)BothObesity details
Associated Disorders and Diseases
Disease References
Obesity
  1. Serlie MJ, Meijer AJ, Groener JE, Duran M, Endert E, Fliers E, Aerts JM, Sauerwein HP: Short-term manipulation of plasma free fatty acids does not change skeletal muscle concentrations of ceramide and glucosylceramide in lean and overweight subjects. J Clin Endocrinol Metab. 2007 Apr;92(4):1524-9. Epub 2007 Jan 30. [17264178 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB023556
KNApSAcK IDNot Available
Chemspider ID16744954
KEGG Compound IDC01190
BioCyc IDGLUCOSYL_CERAMIDE
BiGG IDNot Available
Wikipedia LinkGlucocerebroside
NuGOwiki LinkHMDB04969
Metagene LinkHMDB04969
METLIN ID7222
PubChem Compound20057354
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Hara A, Kitazawa N, Taketomi T: Abnormalities of glycosphingolipids in mucopolysaccharidosis type III B. J Lipid Res. 1984 Feb;25(2):175-84. [6423755 ]
  2. Beutler E: Gaucher disease. Blood Rev. 1988 Mar;2(1):59-70. [3289655 ]
  3. Kaye EM, Ullman MD, Wilson ER, Barranger JA: Type 2 and type 3 Gaucher disease: a morphological and biochemical study. Ann Neurol. 1986 Aug;20(2):223-30. [3752966 ]
  4. Conradi NG, Kalimo H, Sourander P: Reactions of vessel walls and brain parenchyma to the accumulation of Gaucher cells in the Norrbottnian type (type III) of Gaucher disease. Acta Neuropathol (Berl). 1988;75(4):385-90. [3364161 ]
  5. Smith RL, Hutchins GM, Sack GH Jr, Ridolfi RL: Unusual cardiac, renal and pulmonary involvement in Gaucher's disease. Intersitial glucocerebroside accumulation, pulmonary hypertension and fatal bone marrow embolization. Am J Med. 1978 Aug;65(2):352-60. [686020 ]
  6. Ohashi T: [Gene therapy for Gaucher disease] Nippon Rinsho. 1995 Dec;53(12):3089-94. [8577064 ]
  7. Nishimura RN, Barranger JA: Neurologic complications of Gaucher's disease, type 3. Arch Neurol. 1980 Feb;37(2):92-3. [6766716 ]
  8. Eto Y, Ida H: [Molecular studies of Gaucher disease] Rinsho Byori. 1996 Apr;44(4):327-34. [8847814 ]
  9. Naito M, Takahashi K, Hojo H: An ultrastructural and experimental study on the development of tubular structures in the lysosomes of Gaucher cells. Lab Invest. 1988 May;58(5):590-8. [3367638 ]
  10. Mariani G, Filocamo M, Giona F, Villa G, Amendola A, Erba P, Buffoni F, Copello F, Pierini A, Minichilli F, Gatti R, Brady RO: Severity of bone marrow involvement in patients with Gaucher's disease evaluated by scintigraphy with 99mTc-sestamibi. J Nucl Med. 2003 Aug;44(8):1253-62. [12902415 ]
  11. Soffer D, Yamanaka T, Wenger DA, Suzuki K, Suzuki K: Central nervous system involvement in adult-onset Gaucher's disease. Acta Neuropathol (Berl). 1980;49(1):1-6. [7355669 ]
  12. Ohashi T: [Gaucher disease] Nippon Rinsho. 1995 Dec;53(12):2943-6. [8577040 ]
  13. Starzl TE, Demetris AJ, Trucco M, Ricordi C, Ildstad S, Terasaki PI, Murase N, Kendall RS, Kocova M, Rudert WA, et al.: Chimerism after liver transplantation for type IV glycogen storage disease and type 1 Gaucher's disease. N Engl J Med. 1993 Mar 18;328(11):745-9. [8437594 ]
  14. Pilz H, Heipertz R: [Differential diagnosis of congenital lipidoses by lipid analyses of body fluids, biopsy and autopsy tissue] Fortschr Neurol Psychiatr Grenzgeb. 1975 Nov;43(11):602-17. [53174 ]
  15. Nilsson O, Grabowski GA, Ludman MD, Desnick RJ, Svennerholm L: Glycosphingolipid studies of visceral tissues and brain from type 1 Gaucher disease variants. Clin Genet. 1985 May;27(5):443-50. [3924448 ]
  16. Harzer K, Massenkeil G, Frohlich E: Concurrent increase of cholesterol, sphingomyelin and glucosylceramide in the spleen from non-neurologic Niemann-Pick type C patients but also patients possibly affected with other lipid trafficking disorders. FEBS Lett. 2003 Feb 27;537(1-3):177-81. [12606053 ]
  17. 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 ]
  18. Adar T, Ben-Ami R, Elstein D, Zimran A, Berliner S, Yedgar S, Barshtein G: Aggregation of red blood cells in patients with Gaucher disease. Br J Haematol. 2006 Aug;134(4):432-7. Epub 2006 Jul 10. [16827817 ]
  19. Dolen EG, Berdon WE, Ruzal-Shapiro C: "Cold bone scans" as a sign of hemorrhagic infarcts of the spine in Gaucher's disease. Pediatr Radiol. 1997 Jun;27(6):514-6. [9174023 ]
  20. Daniels LB, Coyle PJ, Glew RH, Radin NS, Labow RS: Brain glucocerebrosidase in Gaucher's disease. Arch Neurol. 1982 Sep;39(9):550-6. [6810854 ]
  21. Stirnemann J, Belmatoug N: [Adult Gaucher disease] Rev Med Interne. 2001 Dec;22 Suppl 3:374s-383s. [11794882 ]
  22. Erickson JS, Radin NS: N-hexyl-O-glucosyl sphingosine, an inhibitor of glucosyl ceramide -glucosidase. J Lipid Res. 1973 Mar;14(2):133-7. [4698260 ]
  23. Deguchi H, Bouma BN, Middeldorp S, Lee YM, Griffin JH: Decreased plasma sensitivity to activated protein C by oral contraceptives is associated with decreases in plasma glucosylceramide. J Thromb Haemost. 2005 May;3(5):935-8. [15869587 ]
  24. Shoenfeld Y, Gallant LA, Shaklai M, Livni E, Djaldetti M, Pinkhas J: Gaucher's disease: a disease with chronic stimulation of the immune system. Arch Pathol Lab Med. 1982 Aug;106(8):388-91. [7049116 ]
  25. Ringden O, Groth CG, Erikson A, Granqvist S, Mansson JE, Sparrelid E: Ten years' experience of bone marrow transplantation for Gaucher disease. Transplantation. 1995 Mar 27;59(6):864-70. [7701581 ]
  26. Nilsson O, Mansson JE, Hakansson G, Svennerholm L: The occurrence of psychosine and other glycolipids in spleen and liver from the three major types of Gaucher's disease. Biochim Biophys Acta. 1982 Sep 14;712(3):453-63. [7126619 ]
  27. Dann K, Althaus C, Kersten A, vom Dahl S, Sundmacher R: [Uveitis masquerade syndrome in Gaucher disease. Causal treatment by alglucerase substitution therapy] Klin Monatsbl Augenheilkd. 1998 Dec;213(6):358-61. [10048015 ]
  28. Conradi NG, Sourander P, Nilsson O, Svennerholm L, Erikson A: Neuropathology of the Norrbottnian type of Gaucher disease. Morphological and biochemical studies. Acta Neuropathol (Berl). 1984;65(2):99-109. [6524300 ]
  29. Beutler E: Gaucher disease: new molecular approaches to diagnosis and treatment. Science. 1992 May 8;256(5058):794-9. [1589760 ]
  30. Owada M, Sakiyama T, Kitagawa T: Neuropathic Gaucher's disease with normal 4-methylumbelliferyl-beta-glucosidase activity in the liver. Pediatr Res. 1977 May;11(5):641-6. [870871 ]
  31. Nilsson O, Svennerholm L: Accumulation of glucosylceramide and glucosylsphingosine (psychosine) in cerebrum and cerebellum in infantile and juvenile Gaucher disease. J Neurochem. 1982 Sep;39(3):709-18. [7097276 ]
  32. Liu Y, Suzuki K, Reed JD, Grinberg A, Westphal H, Hoffmann A, Doring T, Sandhoff K, Proia RL: Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure. Proc Natl Acad Sci U S A. 1998 Mar 3;95(5):2503-8. [9482915 ]
  33. Poll LW, Maas M, Terk MR, Roca-Espiau M, Bembi B, Ciana G, Weinreb NJ: Response of Gaucher bone disease to enzyme replacement therapy. Br J Radiol. 2002;75 Suppl 1:A25-36. [12036830 ]
  34. Campbell PE, Harris CM, Harris CM, Sirimanna T, Vellodi A: A model of neuronopathic Gaucher disease. J Inherit Metab Dis. 2003;26(7):629-39. [14707511 ]
  35. Schaison G, Caubel I, Belmatoug N, Billette de Villemeur T, Saudubray JM: [French results of enzyme replacement therapy in Gaucher's disease] Bull Acad Natl Med. 2002;186(5):851-61; discussion 861-3. [12412377 ]
  36. Hollak CE, Boot RG, Poorthuis BJ, Aerts JM: [From gene to disease; Gaucher disease] Ned Tijdschr Geneeskd. 2005 Sep 24;149(39):2163-6. [16223076 ]

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 hydrolase activity, hydrolyzing O-glycosyl compounds
Specific function:
LPH splits lactose in the small intestine.
Gene Name:
LCT
Uniprot ID:
P09848
Molecular weight:
218584.77
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:
Involved in hydrolase activity, hydrolyzing O-glycosyl compounds
Specific function:
Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. Isoform 2 has no beta-galactosidase catalytic activity, but plays functional roles in the formation of extracellular elastic fibers (elastogenesis) and in the development of connective tissue. Seems to be identical to the elastin-binding protein (EBP), a major component of the non-integrin cell surface receptor expressed on fibroblasts, smooth muscle cells, chondroblasts, leukocytes, and certain cancer cell types. In elastin producing cells, associates with tropoelastin intracellularly and functions as a recycling molecular chaperone which facilitates the secretions of tropoelastin and its assembly into elastic fibers.
Gene Name:
GLB1
Uniprot ID:
P16278
Molecular weight:
Not Available
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
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 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 protein kinase activity
Specific function:
Responsible for the regulation of fatty acid synthesis by phosphorylation of acetyl-CoA carboxylase. It also regulates cholesterol synthesis via phosphorylation and inactivation of hormone-sensitive lipase and hydroxymethylglutaryl-CoA reductase. Appears to act as a metabolic stress-sensing protein kinase switching off biosynthetic pathways when cellular ATP levels are depleted and when 5'-AMP rises in response to fuel limitation and/or hypoxia. This is a catalytic subunit
Gene Name:
PRKAA1
Uniprot ID:
Q13131
Molecular weight:
64008.6
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:
Required for the biosynthesis of glycosphingolipids.
Gene Name:
B4GALT6
Uniprot ID:
Q9UBX8
Molecular weight:
44913.315
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.