Pathways > Glycosphingolipid catabolism
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Glycosphingolipid catabolism

Proteins (17)

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)
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)
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)
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)
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)
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)

Compounds (12)

CompoundDescription
phosphorylcholineCalcium and magnesium salts used therapeutically in hepatobiliary dysfunction.
hydronium ion
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.
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.
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.
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)
acetylgalactosamineThe N-acetyl derivative of galactosamine.
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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