| 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) |
| 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) |
| 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) |
| 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) |
| 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) |
| 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) |
| Compound | Description |
|---|
| phosphorylcholine | Calcium and magnesium salts used therapeutically in hepatobiliary dysfunction. |
| hydronium ion | |
| 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. |
| 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. |
| 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. |
| 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) |
| acetylgalactosamine | The N-acetyl derivative of galactosamine. |
| 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. |