Pathways > Glycosphingolipid metabolism
Page last updated: 2024-10-06

Glycosphingolipid metabolism

Proteins (29)

ProteinSynonymsTaxonomy
Arylsulfatase AASA; EC 3.1.6.8; Cerebroside-sulfataseHomo sapiens (human)
Lysosomal acid glucosylceramidaseLysosomal acid GCase; EC 3.2.1.45; Acid beta-glucosidase; Alglucerase; Beta-glucocerebrosidase; Beta-GC; Cholesterol glucosyltransferase; SGTase; 2.4.1.-; Cholesteryl-beta-glucosidase; 3.2.1.-; D-glucosyl-N-acylsphingosine glucohydrolase; ImigluceraseHomo sapiens (human)
2-hydroxyacylsphingosine 1-beta-galactosyltransferaseEC 2.4.1.47; Ceramide UDP-galactosyltransferase; Cerebroside synthase; UDP-galactose-ceramide galactosyltransferaseHomo sapiens (human)
Cytosolic beta-glucosidaseEC 3.2.1.21; Cytosolic beta-glucosidase-like protein 1; Cytosolic glycosylceramidase; Cytosolic GCase; Glucosidase beta acid 3; Glucosylceramidase beta 3; Klotho-related protein; KLrPHomo sapiens (human)
Ceramide kinasehCERK; EC 2.7.1.138; Acylsphingosine kinase; Lipid kinase 4; LK4Homo sapiens (human)
Alpha-galactosidase AEC 3.2.1.22; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Galactosylgalactosylglucosylceramidase GLA; 3.2.1.47; MelibiaseHomo sapiens (human)
Cation-dependent mannose-6-phosphate receptorCD Man-6-P receptor; CD-MPR; 46 kDa mannose 6-phosphate receptor; MPR 46Homo sapiens (human)
Sialidase-3EC 3.2.1.18; Ganglioside sialidasedis; Membrane sialidase; N-acetyl-alpha-neuraminidase 3Homo sapiens (human)
Sialidase-2EC 3.2.1.18; Cytosolic sialidase; N-acetyl-alpha-neuraminidase 2Homo sapiens (human)
Sphingomyelin phosphodiesteraseEC 3.1.4.12; Acid sphingomyelinase; aSMaseHomo sapiens (human)
Acid ceramidaseAC; ACDase; Acid CDase; EC 3.5.1.23; Acylsphingosine deacylase; N-acylethanolamine hydrolase ASAH1; 3.5.1.-; N-acylsphingosine amidohydrolase; Putative 32 kDa heart protein; PHP32Homo sapiens (human)
Ceramide glucosyltransferaseEC 2.4.1.80; GLCT-1; Glucosylceramide synthase; GCS; UDP-glucose ceramide glucosyltransferase; UDP-glucose:N-acylsphingosine D-glucosyltransferaseHomo sapiens (human)
Non-lysosomal glucosylceramidaseNLGase; EC 3.2.1.45; Beta-glucocerebrosidase 2; Beta-glucosidase 2; Bile acid beta-glucosidase GBA2; Bile acid glucosyl transferase GBA2; Cholesterol glucosyltransferase GBA2; 2.4.1.-; Cholesteryl-beta-glucosidase GBA2; 3.2.1.-; Glucosylceramidase 2Homo sapiens (human)
Lactosylceramide 4-alpha-galactosyltransferaseEC 2.4.1.228; Alpha-1,4-N-acetylglucosaminyltransferase; Alpha-1,4-galactosyltransferase; Alpha4Gal-T1; CD77 synthase; Globotriaosylceramide synthase; Gb3 synthase; P1/Pk synthase; UDP-galactose:beta-D-galactosyl-beta1-R 4-alpha-D-galactosyltransferaseHomo sapiens (human)
ProsaposinProactivator polypeptideHomo sapiens (human)
Ectonucleotide pyrophosphatase/phosphodiesterase family member 7E-NPP 7; NPP-7; EC 3.1.4.12; Alkaline sphingomyelin phosphodiesterase; Intestinal alkaline sphingomyelinase; Alk-SMaseHomo sapiens (human)
GalactocerebrosidaseGALCERase; EC 3.2.1.46; Galactocerebroside beta-galactosidase; Galactosylceramidase; Galactosylceramide beta-galactosidaseHomo sapiens (human)
Formylglycine-generating enzymeFGE; EC 1.8.3.7; C-alpha-formylglycine-generating enzyme 1; Sulfatase-modifying factor 1Homo sapiens (human)
Galactosylceramide sulfotransferaseGalCer sulfotransferase; EC 2.8.2.11; 3'-phosphoadenosine-5'-phosphosulfate:GalCer sulfotransferase; 3'-phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase; Cerebroside sulfotransferaseHomo sapiens (human)
Beta-1,4 N-acetylgalactosaminyltransferase 1EC 2.4.1.92; (N-acetylneuraminyl)-galactosylglucosylceramide; GM2/GD2 synthase; GalNAc-THomo sapiens (human)
Beta-1,3-galactosyltransferase 4Beta-1,3-GalTase 4; Beta3Gal-T4; Beta3GalT4; GalT4; b3Gal-T4; EC 2.4.1.62; Gal-T2; Ganglioside galactosyltransferase; UDP-galactose:beta-N-acetyl-galactosamine-beta-1,3-galactosyltransferaseHomo sapiens (human)
Lactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferaseEC 2.4.1.206; Lactotriaosylceramide synthase; Lc(3)Cer synthase; Lc3 synthase; UDP-GlcNAc:beta-Gal beta-1,3-N-acetylglucosaminyltransferase 5; BGnT-5; Beta-1,3-Gn-T5; Beta-1,3-N-acetylglucosaminyltransferase 5; Beta3Gn-T5Homo sapiens (human)
UDP-GalNAc:beta-1,3-N-acetylgalactosaminyltransferase 1Beta-1,3-GalNAc-T1; EC 2.4.1.79; Beta-1,3-galactosyltransferase 3; Beta-1,3-GalTase 3; Beta3Gal-T3; Beta3GalT3; b3Gal-T3; Beta-3-Gx-T3; Galactosylgalactosylglucosylceramide beta-D-acetyl-galactosaminyltransferase; Globoside synthase; UDP-N-acetylgalactosaHomo sapiens (human)
Beta-1,4-galactosyltransferase 5Beta-1,4-GalTase 5; Beta4Gal-T5; b4Gal-T5; EC 2.4.1.-; Beta-1,4-GalT II; Glucosylceramide beta-1,4-galactosyltransferase; 2.4.1.274; Lactosylceramide synthase; LacCer synthase; UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 5; UDP-galactose:beta-N-aceHomo sapiens (human)
Ganglioside GM2 activatorCerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3Homo sapiens (human)
Inactive C-alpha-formylglycine-generating enzyme 2Paralog of formylglycine-generating enzyme; pFGE; Sulfatase-modifying factor 2Homo sapiens (human)
Sphingomyelin phosphodiesterase 4EC 3.1.4.12; Neutral sphingomyelinase 3; nSMase-3; nSMase3; Neutral sphingomyelinase IIIHomo sapiens (human)
Beta-1,4-galactosyltransferase 6Beta-1,4-GalTase 6; Beta4Gal-T6; b4Gal-T6; EC 2.4.1.-; Glucosylceramide beta-1,4-galactosyltransferase; 2.4.1.274; Lactosylceramide synthase; LacCer synthase; UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 6; UDP-Gal:glucosylceramide beta-1,4-galactosHomo sapiens (human)
Lactosylceramide alpha-2,3-sialyltransferaseEC 2.4.99.9; CMP-NeuAc:lactosylceramide alpha-2,3-sialyltransferase; GM3 synthase; Ganglioside GM3 synthase; ST3Gal V; ST3GalV; Sialyltransferase 9Homo sapiens (human)

Compounds (21)

CompoundDescription
phosphorylcholineCalcium and magnesium salts used therapeutically in hepatobiliary dysfunction.
hydronium ion
uridine diphosphateA uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety.
NADH
CalciumA basic element found in nearly all tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.
ManganeseA trace element with atomic symbol Mn, atomic number 25, and atomic weight 54.94. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver. (From AMA Drug Evaluations Annual 1992, p2035)
PhosphorylcholineCalcium and magnesium salts used therapeutically in hepatobiliary dysfunction.
galactoseAn aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. Deficiency of galactosyl-1-phosphate uridyltransferase (GALACTOSE-1-PHOSPHATE URIDYL-TRANSFERASE DEFICIENCY DISEASE) causes an error in galactose metabolism called GALACTOSEMIA, resulting in elevations of galactose in the blood.
uridine diphosphateA uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
MagnesiumA metallic element that has the atomic symbol Mg, atomic number 12, and atomic weight 24.31. It is important for the activity of many enzymes, especially those involved in OXIDATIVE PHOSPHORYLATION.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
G(M1) GangliosideA specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis.
Guanosine DiphosphateA guanine nucleotide containing two phosphate groups esterified to the sugar moiety.
nadA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
adenosine triphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
Cytidine MonophosphateCytidine (dihydrogen phosphate). A cytosine nucleotide containing one phosphate group esterified to the sugar moiety in the 2', 3' or 5' position.
adenosine 3'-phosphate-5'-phosphate
acetylgalactosamineThe N-acetyl derivative of galactosamine.
uridine diphosphate n-acetylgalactosamineA nucleoside diphosphate sugar which serves as a source of N-acetylgalactosamine for glycoproteins, sulfatides and cerebrosides.
glucoseA primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement.
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