1.02011-09-21 01:31:03 UTC2015-07-21 06:59:15 UTCFDB027859Galactosylceramide (d18:1/18:0)GGalactosylceramide (GalCer) is a non-acidic monoglycosphingolipid, i.e. a sphingolipid with one carbohydrate moiety attached to a ceramide unit. It is 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. [HMDB]N-(octadecanoyl)-1-b-galactosyl-sphing-4-enineN-(octadecanoyl)-1-beta-galactosyl-sphing-4-enineC42H81NO8728.0944727.596218573N-(3-hydroxy-1-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl)octadecanamideN-(3-hydroxy-1-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl)octadecanamide[H][C@]1(CO)O[C@@]([H])(OCC(NC(=O)CCCCCCCCCCCCCCCCC)C(O)C=CCCCCCCCCCCCCC)[C@]([H])(O)[C@@]([H])(O)[C@@]1([H])OInChI=1S/C42H81NO8/c1-3-5-7-9-11-13-15-17-18-20-22-24-26-28-30-32-38(46)43-35(34-50-42-41(49)40(48)39(47)37(33-44)51-42)36(45)31-29-27-25-23-21-19-16-14-12-10-8-6-4-2/h29,31,35-37,39-42,44-45,47-49H,3-28,30,32-34H2,1-2H3,(H,43,46)/t35?,36?,37-,39+,40+,41-,42-/m1/s1YMYQEDCYNANIPI-DLQUMWEOSA-N 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.Glycosyl-N-acylsphingosinesOrganic compoundsLipids and lipid-like moleculesSphingolipidsGlycosphingolipidsAliphatic heteromonocyclic compoundsAcetalsAlkyl glycosidesCarbonyl compoundsFatty acyl glycosides of mono- and disaccharidesHexosesHydrocarbon derivativesN-acyl aminesO-glycosyl compoundsOrganic oxidesOrganonitrogen compoundsOrganopnictogen compoundsOxacyclic compoundsOxanesPolyolsPrimary alcoholsSecondary alcoholsSecondary carboxylic acid amidesAcetalAlcoholAliphatic heteromonocyclic compoundAlkyl glycosideCarbonyl groupCarboxamide groupCarboxylic acid derivativeFatty acylFatty acyl glycosideFatty acyl glycoside of mono- or disaccharideFatty amideGlycosyl compoundGlycosyl-n-acylsphingosineHexose monosaccharideHydrocarbon derivativeMonosaccharideN-acyl-amineO-glycosyl compoundOrganic nitrogen compoundOrganic oxideOrganic oxygen compoundOrganoheterocyclic compoundOrganonitrogen compoundOrganooxygen compoundOrganopnictogen compoundOxacycleOxanePolyolPrimary alcoholSecondary alcoholSecondary carboxylic acid amideSolidlogp8.13logs-6.00solubility7.23e-04 g/llogp9.98pka_strongest_acidic12.18pka_strongest_basic0.019iupacN-(3-hydroxy-1-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}octadec-4-en-2-yl)octadecanamideaverage_mass728.0944mono_mass727.596218573smiles[H][C@]1(CO)O[C@@]([H])(OCC(NC(=O)CCCCCCCCCCCCCCCCC)C(O)C=CCCCCCCCCCCCCC)[C@]([H])(O)[C@@]([H])(O)[C@@]1([H])OformulaC42H81NO8inchiInChI=1S/C42H81NO8/c1-3-5-7-9-11-13-15-17-18-20-22-24-26-28-30-32-38(46)43-35(34-50-42-41(49)40(48)39(47)37(33-44)51-42)36(45)31-29-27-25-23-21-19-16-14-12-10-8-6-4-2/h29,31,35-37,39-42,44-45,47-49H,3-28,30,32-34H2,1-2H3,(H,43,46)/t35?,36?,37-,39+,40+,41-,42-/m1/s1inchikeyYMYQEDCYNANIPI-DLQUMWEOSA-Npolar_surface_area148.71refractivity207.39polarizability92.8rotatable_bond_count35acceptor_count8donor_count6physiological_charge0formal_charge0HMDB10709#<Reference:0x00005556760918a8>#<Reference:0x00005556760916c8>#<Reference:0x0000555676091510>#<Reference:0x0000555676091358>#<Reference:0x0000555676091150>#<Reference:0x0000555676090ef8>#<Reference:0x0000555676090cf0>#<Reference:0x00005556760909d0>#<Reference:0x0000555676090778>#<Reference:0x0000555676090598>#<Reference:0x0000555676090138>#<Reference:0x000055567608ff08>#<Reference:0x000055567608fd50>#<Reference:0x000055567608fb98>AnatidaeType 1specificAnatidae8830BeefaloType 1specificBos taurus X Bison bison297284BisonType 1specificBison bison9901BuffaloType 1specificBubalus bubalis89462Cattle (Beef, Veal)Type 1specificBos taurus9913ChickenType 1specificGallus gallus9031Columbidae (Dove, Pigeon)Type 1specificColumbidae8930DeerType 1specificCervidae9850Domestic goatType 1specificCapra aegagrus hircus9925Domestic pigType 1specificSus scrofa domestica9825ElkType 1specificCervus canadensis1574408EmuType 1specificDromaius novaehollandiae8790European rabbitType 1specificOryctolagus9984Greylag gooseType 1specificAnser anser8843Guinea henType 1specificNumida meleagris8996HorseType 1specificEquus caballus9796Mallard duckType 1specificAnas platyrhynchos8839Mountain hareType 1specificLepus timidus62621Mule deerType 1specificOdocoileus9871OstrichType 1specificStruthio camelus8801PheasantType 1specificPhasianus colchicus9054QuailType 1specificPhasianidae9005RabbitType 1specificLeporidae9979Rock ptarmiganType 1specificLagopus muta64668Sheep (Mutton, Lamb)Type 1specificOvis aries9940SquabType 1specificColumba8931TurkeyType 1specificMeleagris gallopavo9103Velvet duckType 1specificMelanitta fusca371864Wild boarType 1specificSus scrofa98232-hydroxyacylsphingosine 1-beta-galactosyltransferaseQ16880UGT8Acid ceramidaseQ13510ASAH1Alkaline ceramidase 1Q8TDN7ACER1Alkaline ceramidase 2Q5QJU3ACER2Alpha-galactosidase AP06280GLAAntigen-presenting glycoprotein CD1dP15813CD1DBeta-1,3-galactosyltransferase 5Q9Y2C3B3GALT5Ceramide glucosyltransferaseQ16739UGCGCeramide synthase 1P27544CERS1Collagen type IV alpha-3-binding proteinQ9Y5P4COL4A3BPEpididymal secretory protein E1P61916NPC2GalactocerebrosidaseP54803GALCGalactosylceramide sulfotransferaseQ99999GAL3ST1Ganglioside GM2 activatorP17900GM2AGlucosylceramidaseP04062GBAGPI ethanolamine phosphate transferase 1O95427PIGNGPI ethanolamine phosphate transferase 2Q5H8A4PIGGGPI ethanolamine phosphate transferase 3Q8TEQ8PIGOGPI mannosyltransferase 1Q9H3S5PIGMGPI mannosyltransferase 2Q9NUD9PIGVGPI mannosyltransferase 3Q92521PIGBGPI mannosyltransferase 4Q86VD9PIGZGPI transamidase component PIG-SQ96S52PIGSGPI transamidase component PIG-TQ969N2PIGTGPI-anchor transamidaseQ92643PIGKLactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferaseQ9BYG0B3GNT5Lactosylceramide 4-alpha-galactosyltransferaseQ9NPC4A4GALTN-acetylglucosaminyl-phosphatidylinositol de-N-acetylaseQ9Y2B2PIGLNeutral ceramidaseQ9NR71ASAH2Non-lysosomal glucosylceramidaseQ9HCG7GBA2Phosphatidylinositol glycan anchor biosynthesis class U proteinQ9H490PIGUPhosphatidylinositol N-acetylglucosaminyltransferase subunit AP37287PIGAPhosphatidylinositol N-acetylglucosaminyltransferase subunit CQ92535PIGCPhosphatidylinositol N-acetylglucosaminyltransferase subunit HQ14442PIGHPhosphatidylinositol N-acetylglucosaminyltransferase subunit PP57054PIGPPhosphatidylinositol N-acetylglucosaminyltransferase subunit QQ9BRB3PIGQPhosphatidylinositol N-acetylglucosaminyltransferase subunit YQ3MUY2PIGYPhosphatidylinositol-glycan biosynthesis class F proteinQ07326PIGFPhosphatidylinositol-glycan biosynthesis class W proteinQ7Z7B1PIGWPhosphatidylinositol-glycan biosynthesis class X proteinQ8TBF5PIGXPutative neutral ceramidase CP0C7U2ASAH2CSialidase-2Q9Y3R4NEU2Sialidase-4Q8WWR8NEU4Sphingomyelin phosphodiesteraseP17405SMPD1T-cell surface glycoprotein CD1e, membrane-associatedP15812CD1E