| Protein | Synonyms | Taxonomy |
|---|
| 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) |
| 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) |
| 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) |
| Sphingomyelin phosphodiesterase | EC 3.1.4.12; Acid sphingomyelinase; aSMase | 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) |
| 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) |
| Prosaposin | Proactivator polypeptide | 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) |
| 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) |
| 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) |
| 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) |
| Inactive C-alpha-formylglycine-generating enzyme 2 | Paralog of formylglycine-generating enzyme; pFGE; Sulfatase-modifying factor 2 | 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. |
| 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) |
| Phosphorylcholine | Calcium and magnesium salts used therapeutically in hepatobiliary dysfunction. |
| 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) |
| 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. |
| 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) |
| 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. |
| adenosine 3'-phosphate-5'-phosphate | |
| acetylgalactosamine | The N-acetyl derivative of galactosamine. |
| 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. |