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

Sphingolipid metabolism

Proteins (52)

ProteinSynonymsTaxonomy
Alkaline ceramidase 2AlkCDase 2; Alkaline CDase 2; haCER2; EC 3.5.1.-; EC 3.5.1.23; Acylsphingosine deacylase 3-like; N-acylsphingosine amidohydrolase 3-likeHomo sapiens (human)
Fatty acid 2-hydroxylaseEC 1.14.18.-; Fatty acid alpha-hydroxylase; Fatty acid hydroxylase domain-containing protein 1Homo sapiens (human)
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)
Sphingosine kinase 1SK 1; SPK 1; EC 2.7.1.91; Acetyltransferase SPHK1; 2.3.1.-Homo sapiens (human)
Cytochrome b5 type BCytochrome b5 outer mitochondrial membrane isoformHomo 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)
Serine palmitoyltransferase 1EC 2.3.1.50; Long chain base biosynthesis protein 1; LCB 1; Serine-palmitoyl-CoA transferase 1; SPT 1; SPT1Homo sapiens (human)
Serine palmitoyltransferase 2EC 2.3.1.50; Long chain base biosynthesis protein 2; LCB 2; Long chain base biosynthesis protein 2a; LCB2a; Serine-palmitoyl-CoA transferase 2; SPT 2Homo 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)
Sphingolipid delta(4)-desaturase DES1EC 1.14.19.17; Cell migration-inducing gene 15 protein; Degenerative spermatocyte homolog 1; Dihydroceramide desaturase-1; Membrane lipid desaturaseHomo sapiens (human)
Oxysterol-binding protein 1Homo sapiens (human)
Sphingomyelin phosphodiesteraseEC 3.1.4.12; Acid sphingomyelinase; aSMaseHomo sapiens (human)
Casein kinase I isoform gamma-2CKI-gamma 2; EC 2.7.11.1Homo 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)
Sphingosine kinase 2SK 2; SPK 2; EC 2.7.1.91Homo sapiens (human)
Sphingosine-1-phosphate lyase 1S1PL; SP-lyase 1; SPL 1; hSPL; EC 4.1.2.27; Sphingosine-1-phosphate aldolaseHomo sapiens (human)
Phosphatidylcholine:ceramide cholinephosphotransferase 2EC 2.7.8.27; Sphingomyelin synthase 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)
3-ketodihydrosphingosine reductaseKDS reductase; EC 1.1.1.102; 3-dehydrosphinganine reductase; Follicular variant translocation protein 1; FVT-1; Short chain dehydrogenase/reductase family 35C member 1Homo sapiens (human)
Alkaline ceramidase 1AlkCDase 1; Alkaline CDase 1; EC 3.5.1.-; EC 3.5.1.23; Acylsphingosine deacylase 3; N-acylsphingosine amidohydrolase 3Homo sapiens (human)
ProsaposinProactivator polypeptideHomo sapiens (human)
Phosphatidylcholine:ceramide cholinephosphotransferase 1 EC 2.7.8.27; Medulla oblongata-derived protein; Protein Mob; Sphingomyelin synthase 1; Transmembrane protein 23Homo sapiens (human)
Aldehyde dehydrogenase family 3 member B2EC 1.2.1.3; Aldehyde dehydrogenase 8Homo 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)
Serine palmitoyltransferase 3EC 2.3.1.50; Long chain base biosynthesis protein 2b; LCB2b; Long chain base biosynthesis protein 3; LCB 3; Serine-palmitoyl-CoA transferase 3; SPT 3Homo 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)
Sphingomyelin synthase-related protein 1SMSr; EC 2.7.8.-; Ceramide phosphoethanolamine synthase; CPE synthase; Sterile alpha motif domain-containing protein 8; SAM domain-containing protein 8Homo 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)
Aldehyde dehydrogenase family 3 member B1EC 1.2.1.28; EC 1.2.1.5; EC 1.2.1.7; Aldehyde dehydrogenase 7Homo sapiens (human)
Phospholipid phosphatase 1EC 3.1.3.-; EC 3.1.3.106; EC 3.1.3.4; EC 3.1.3.81; Lipid phosphate phosphohydrolase 1; PAP2-alpha; Phosphatidate phosphohydrolase type 2a; Phosphatidic acid phosphatase 2a; PAP-2a; PAP2aHomo sapiens (human)
Sphingolipid delta(4)-desaturase/C4-monooxygenase DES2EC 1.14.18.5; EC 1.14.19.17; Degenerative spermatocyte homolog 2; Sphingolipid 4-desaturase; Sphingolipid C4-monooxygenaseHomo 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)
Protein phosphatase 1LEC 3.1.3.16; Protein phosphatase 1-like; Protein phosphatase 2C isoform epsilon; PP2C-epsilonHomo sapiens (human)
Inactive C-alpha-formylglycine-generating enzyme 2Paralog of formylglycine-generating enzyme; pFGE; Sulfatase-modifying factor 2Homo sapiens (human)
Alkaline ceramidase 3AlkCDase 3; Alkaline CDase 3; EC 3.5.1.-; EC 3.5.1.23; Alkaline dihydroceramidase SB89; Alkaline phytoceramidase; aPHCHomo 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 (37)

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.
ironA metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
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)
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
IronA metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
ZincA metallic element of atomic number 30 and atomic weight 65.38. It is a necessary trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. Zinc deficiency is associated with ANEMIA, short stature, HYPOGONADISM, impaired WOUND HEALING, and geophagia. It is known by the symbol Zn.
PhosphorylcholineCalcium and magnesium salts used therapeutically in hepatobiliary dysfunction.
Orthophosphate
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)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
palmitic acidA common saturated fatty acid found in fats and waxes including olive oil, palm oil, and body lipids.
phytosphingosinediffer with an additional hydroxyl at C-4 & no double bond between C-4 & C-5
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.
phosphorylethanolamineRN given refers to parent cpd; structure
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)
NADPNicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)
NADPNicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)
2-hexadecenal
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.
hexadecanal
adenosine 3'-phosphate-5'-phosphate
Coenzyme A
acetylgalactosamineThe N-acetyl derivative of galactosamine.
SphingosineAn amino alcohol with a long unsaturated hydrocarbon chain. Sphingosine and its derivative sphinganine are the major bases of the sphingolipids in mammals. (Dorland, 28th ed)
sphingosine 1-phosphateRN given refers to (R-(R*,S*-(E)))-isomer; RN for cpd without isomeric designation not available 8/89
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.
chemdatabank.com