| Protein | Synonyms | Taxonomy |
|---|
| Alkaline ceramidase 2 | AlkCDase 2; Alkaline CDase 2; haCER2; EC 3.5.1.-; EC 3.5.1.23; Acylsphingosine deacylase 3-like; N-acylsphingosine amidohydrolase 3-like | Homo sapiens (human) |
| Fatty acid 2-hydroxylase | EC 1.14.18.-; Fatty acid alpha-hydroxylase; Fatty acid hydroxylase domain-containing protein 1 | Homo sapiens (human) |
| Arylsulfatase A | ASA; EC 3.1.6.8; Cerebroside-sulfatase | Homo sapiens (human) |
| Lysosomal acid glucosylceramidase | Lysosomal 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; Imiglucerase | Homo sapiens (human) |
| Sphingosine kinase 1 | SK 1; SPK 1; EC 2.7.1.91; Acetyltransferase SPHK1; 2.3.1.- | Homo sapiens (human) |
| Cytochrome b5 type B | Cytochrome b5 outer mitochondrial membrane isoform | Homo sapiens (human) |
| 2-hydroxyacylsphingosine 1-beta-galactosyltransferase | EC 2.4.1.47; Ceramide UDP-galactosyltransferase; Cerebroside synthase; UDP-galactose-ceramide galactosyltransferase | Homo sapiens (human) |
| Cytosolic beta-glucosidase | EC 3.2.1.21; Cytosolic beta-glucosidase-like protein 1; Cytosolic glycosylceramidase; Cytosolic GCase; Glucosidase beta acid 3; Glucosylceramidase beta 3; Klotho-related protein; KLrP | Homo sapiens (human) |
| Serine palmitoyltransferase 1 | EC 2.3.1.50; Long chain base biosynthesis protein 1; LCB 1; Serine-palmitoyl-CoA transferase 1; SPT 1; SPT1 | Homo sapiens (human) |
| Serine palmitoyltransferase 2 | EC 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 2 | Homo sapiens (human) |
| Ceramide kinase | hCERK; EC 2.7.1.138; Acylsphingosine kinase; Lipid kinase 4; LK4 | Homo sapiens (human) |
| Alpha-galactosidase A | EC 3.2.1.22; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Galactosylgalactosylglucosylceramidase GLA; 3.2.1.47; Melibiase | Homo sapiens (human) |
| Cation-dependent mannose-6-phosphate receptor | CD Man-6-P receptor; CD-MPR; 46 kDa mannose 6-phosphate receptor; MPR 46 | Homo sapiens (human) |
| Sialidase-3 | EC 3.2.1.18; Ganglioside sialidasedis; Membrane sialidase; N-acetyl-alpha-neuraminidase 3 | Homo sapiens (human) |
| Sialidase-2 | EC 3.2.1.18; Cytosolic sialidase; N-acetyl-alpha-neuraminidase 2 | Homo sapiens (human) |
| Sphingolipid delta(4)-desaturase DES1 | EC 1.14.19.17; Cell migration-inducing gene 15 protein; Degenerative spermatocyte homolog 1; Dihydroceramide desaturase-1; Membrane lipid desaturase | Homo sapiens (human) |
| Oxysterol-binding protein 1 | | Homo sapiens (human) |
| Sphingomyelin phosphodiesterase | EC 3.1.4.12; Acid sphingomyelinase; aSMase | Homo sapiens (human) |
| Casein kinase I isoform gamma-2 | CKI-gamma 2; EC 2.7.11.1 | Homo sapiens (human) |
| Acid ceramidase | AC; 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; PHP32 | Homo sapiens (human) |
| Ceramide glucosyltransferase | EC 2.4.1.80; GLCT-1; Glucosylceramide synthase; GCS; UDP-glucose ceramide glucosyltransferase; UDP-glucose:N-acylsphingosine D-glucosyltransferase | Homo sapiens (human) |
| Non-lysosomal glucosylceramidase | NLGase; 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 2 | Homo sapiens (human) |
| Sphingosine kinase 2 | SK 2; SPK 2; EC 2.7.1.91 | Homo sapiens (human) |
| Sphingosine-1-phosphate lyase 1 | S1PL; SP-lyase 1; SPL 1; hSPL; EC 4.1.2.27; Sphingosine-1-phosphate aldolase | Homo sapiens (human) |
| Phosphatidylcholine:ceramide cholinephosphotransferase 2 | EC 2.7.8.27; Sphingomyelin synthase 2 | Homo sapiens (human) |
| Lactosylceramide 4-alpha-galactosyltransferase | EC 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-galactosyltransferase | Homo sapiens (human) |
| 3-ketodihydrosphingosine reductase | KDS reductase; EC 1.1.1.102; 3-dehydrosphinganine reductase; Follicular variant translocation protein 1; FVT-1; Short chain dehydrogenase/reductase family 35C member 1 | Homo sapiens (human) |
| Alkaline ceramidase 1 | AlkCDase 1; Alkaline CDase 1; EC 3.5.1.-; EC 3.5.1.23; Acylsphingosine deacylase 3; N-acylsphingosine amidohydrolase 3 | Homo sapiens (human) |
| Prosaposin | Proactivator polypeptide | Homo sapiens (human) |
| Phosphatidylcholine:ceramide cholinephosphotransferase 1 | EC 2.7.8.27; Medulla oblongata-derived protein; Protein Mob; Sphingomyelin synthase 1; Transmembrane protein 23 | Homo sapiens (human) |
| Aldehyde dehydrogenase family 3 member B2 | EC 1.2.1.3; Aldehyde dehydrogenase 8 | Homo sapiens (human) |
| Ectonucleotide pyrophosphatase/phosphodiesterase family member 7 | E-NPP 7; NPP-7; EC 3.1.4.12; Alkaline sphingomyelin phosphodiesterase; Intestinal alkaline sphingomyelinase; Alk-SMase | Homo sapiens (human) |
| Galactocerebrosidase | GALCERase; EC 3.2.1.46; Galactocerebroside beta-galactosidase; Galactosylceramidase; Galactosylceramide beta-galactosidase | Homo sapiens (human) |
| Serine palmitoyltransferase 3 | EC 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 3 | Homo sapiens (human) |
| Formylglycine-generating enzyme | FGE; EC 1.8.3.7; C-alpha-formylglycine-generating enzyme 1; Sulfatase-modifying factor 1 | Homo sapiens (human) |
| Galactosylceramide sulfotransferase | GalCer sulfotransferase; EC 2.8.2.11; 3'-phosphoadenosine-5'-phosphosulfate:GalCer sulfotransferase; 3'-phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase; Cerebroside sulfotransferase | Homo sapiens (human) |
| Beta-1,4 N-acetylgalactosaminyltransferase 1 | EC 2.4.1.92; (N-acetylneuraminyl)-galactosylglucosylceramide; GM2/GD2 synthase; GalNAc-T | Homo sapiens (human) |
| Sphingomyelin synthase-related protein 1 | SMSr; EC 2.7.8.-; Ceramide phosphoethanolamine synthase; CPE synthase; Sterile alpha motif domain-containing protein 8; SAM domain-containing protein 8 | Homo sapiens (human) |
| Beta-1,3-galactosyltransferase 4 | Beta-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-galactosyltransferase | Homo sapiens (human) |
| Lactosylceramide 1,3-N-acetyl-beta-D-glucosaminyltransferase | EC 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-T5 | Homo sapiens (human) |
| UDP-GalNAc:beta-1,3-N-acetylgalactosaminyltransferase 1 | Beta-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-acetylgalactosa | Homo sapiens (human) |
| Aldehyde dehydrogenase family 3 member B1 | EC 1.2.1.28; EC 1.2.1.5; EC 1.2.1.7; Aldehyde dehydrogenase 7 | Homo sapiens (human) |
| Phospholipid phosphatase 1 | EC 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; PAP2a | Homo sapiens (human) |
| Sphingolipid delta(4)-desaturase/C4-monooxygenase DES2 | EC 1.14.18.5; EC 1.14.19.17; Degenerative spermatocyte homolog 2; Sphingolipid 4-desaturase; Sphingolipid C4-monooxygenase | Homo sapiens (human) |
| Beta-1,4-galactosyltransferase 5 | Beta-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-ace | Homo sapiens (human) |
| Ganglioside GM2 activator | Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3 | Homo sapiens (human) |
| Protein phosphatase 1L | EC 3.1.3.16; Protein phosphatase 1-like; Protein phosphatase 2C isoform epsilon; PP2C-epsilon | Homo sapiens (human) |
| Inactive C-alpha-formylglycine-generating enzyme 2 | Paralog of formylglycine-generating enzyme; pFGE; Sulfatase-modifying factor 2 | Homo sapiens (human) |
| Alkaline ceramidase 3 | AlkCDase 3; Alkaline CDase 3; EC 3.5.1.-; EC 3.5.1.23; Alkaline dihydroceramidase SB89; Alkaline phytoceramidase; aPHC | Homo sapiens (human) |
| Sphingomyelin phosphodiesterase 4 | EC 3.1.4.12; Neutral sphingomyelinase 3; nSMase-3; nSMase3; Neutral sphingomyelinase III | Homo sapiens (human) |
| Beta-1,4-galactosyltransferase 6 | Beta-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-galactos | Homo sapiens (human) |
| Lactosylceramide alpha-2,3-sialyltransferase | EC 2.4.99.9; CMP-NeuAc:lactosylceramide alpha-2,3-sialyltransferase; GM3 synthase; Ganglioside GM3 synthase; ST3Gal V; ST3GalV; Sialyltransferase 9 | Homo sapiens (human) |
| Compound | Description |
|---|
| phosphorylcholine | Calcium and magnesium salts used therapeutically in hepatobiliary dysfunction. |
| hydronium ion | |
| uridine diphosphate | A uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety. |
| NADH | |
| Calcium | A 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. |
| iron | A 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. |
| Manganese | A 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 Dioxide | A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. |
| Iron | A 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. |
| Zinc | A 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. |
| Phosphorylcholine | Calcium and magnesium salts used therapeutically in hepatobiliary dysfunction. |
| Orthophosphate | |
| galactose | An 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 diphosphate | A uracil nucleotide containing a pyrophosphate group esterified to C5 of the sugar moiety. |
| adenosine diphosphate | Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position. |
| Magnesium | A 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. |
| Water | A 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) |
| Oxygen | An 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 acid | A common saturated fatty acid found in fats and waxes including olive oil, palm oil, and body lipids. |
| phytosphingosine | differ with an additional hydroxyl at C-4 & no double bond between C-4 & C-5 |
| G(M1) Ganglioside | A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis. |
| Guanosine Diphosphate | A guanine nucleotide containing two phosphate groups esterified to the sugar moiety. |
| phosphorylethanolamine | RN given refers to parent cpd; structure |
| nad | A 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) |
| NADP | Nicotinamide 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) |
| NADP | Nicotinamide 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 triphosphate | An 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 Monophosphate | Cytidine (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 | |
| acetylgalactosamine | The N-acetyl derivative of galactosamine. |
| Sphingosine | An 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-phosphate | RN given refers to (R-(R*,S*-(E)))-isomer; RN for cpd without isomeric designation not available 8/89 |
| uridine diphosphate n-acetylgalactosamine | A nucleoside diphosphate sugar which serves as a source of N-acetylgalactosamine for glycoproteins, sulfatides and cerebrosides. |
| glucose | A 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. |