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Literature summary extracted from

  • Shayman, J.A.; Abe, A.
    Glucosylceramide synthase: assay and properties (2000), Methods Enzymol., 311, 42-49.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
2.4.1.80 cycloserine induction Canis lupus familiaris
2.4.1.80 NAD+ stimulation, best at 2 mM Homo sapiens
2.4.1.80 NADH stimulation Homo sapiens
2.4.1.80 NADP+ stimulation Homo sapiens
2.4.1.80 NADPH stimulation Homo sapiens

Application

EC Number Application Comment Organism
2.4.1.80 medicine potential therapeutic target for inherited sphingolipidoses such as Gaucher, Fabry, and Tay-Sachs disease Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.4.1.80 cloning and transient expression in GM-95 cell line, mutant cell line containing no enzyme activity, derived from melanoma mutant cell line B16, DNA sequence determination and analysis Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
2.4.1.80 CDP-glucose slight inhibition Homo sapiens
2.4.1.80 Cu2+
-
 Homo sapiens
2.4.1.80 D-threo-1-phenyl-2-(decanoylamino)-3-morpholino-1-propanol-HCl in vivo depletion of enzyme and accumulation of ceramide; not DL-diastereomer Canis lupus familiaris
2.4.1.80 D-threo-1-phenyl-2-(decanoylamino)-3-morpholino-1-propanol-HCl
-
 Homo sapiens
2.4.1.80 Fe2+
-
 Homo sapiens
2.4.1.80 Fe3+
-
 Homo sapiens
2.4.1.80 additional information induction of enzyme by inhibitors in presence of higher ceramide levels Canis lupus familiaris
2.4.1.80 additional information induction of enzyme by inhibitors in presence of higher ceramide levels; no inhibition by conduritol B epoxide Homo sapiens
2.4.1.80 N-butyldeoxynojirimycin
-
 Canis lupus familiaris
2.4.1.80 N-butyldeoxynojirimycin nonspecific inhibitor Homo sapiens
2.4.1.80 Tris 58% loss of activity at 0.2 M Homo sapiens
2.4.1.80 Zn2+
-
 Homo sapiens

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
2.4.1.80 Ca2+ stimulates Homo sapiens
2.4.1.80 Mg2+ stimulates Homo sapiens
2.4.1.80 Mn2+ stimulates Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
2.4.1.80 448530
-
 x * 448530, amino acid sequence determination Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2.4.1.80 UDP-glucose + N-acylsphingosine Canis lupus familiaris
-
 UDP + D-glucosyl-N-acylsphingosine
-
 ?
2.4.1.80 UDP-glucose + N-acylsphingosine Homo sapiens enzyme is involved in renal growth, neuronal differentiation, the establishment of the water permeability barrier in keratinocytes, and multidrug resistance in cancer cell UDP + D-glucosyl-N-acylsphingosine
-
 ?
2.4.1.80 UDP-glucose + N-acylsphingosine Homo sapiens enzyme is involved in renal growth, neuronal differentiation, the establishment of the water permeability barrier in keratinocytes, and multidrug resistance in cancer cell UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?

Organism

EC Number Organism UniProt Comment Textmining
2.4.1.80 Canis lupus familiaris
-
 MDCK cells
-
2.4.1.80 Homo sapiens
-
-
-
2.4.1.80 Homo sapiens Q16739
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.4.1.80 brain
-
 Homo sapiens
-
2.4.1.80 keratinocyte
-
 Homo sapiens
-
2.4.1.80 kidney
-
 Homo sapiens
-
2.4.1.80 MDCK cell
-
 Canis lupus familiaris
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.4.1.80 additional information octanoyl dihydrosphingosine, decanoyl sphingosine, and stearoyl sphingosine are poor substrates Homo sapiens ?
-
 ?
2.4.1.80 UDP-glucose + N-acylsphingosine
-
 Canis lupus familiaris UDP + D-glucosyl-N-acylsphingosine
-
 ?
2.4.1.80 UDP-glucose + N-acylsphingosine acceptor substrate specificity, overview Homo sapiens UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
2.4.1.80 UDP-glucose + N-acylsphingosine i.e. ceramide Homo sapiens UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
2.4.1.80 UDP-glucose + N-acylsphingosine i.e. ceramide Canis lupus familiaris UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
2.4.1.80 UDP-glucose + N-acylsphingosine overview: short-chain ceramide substrates Homo sapiens UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
2.4.1.80 UDP-glucose + N-acylsphingosine enzyme is involved in renal growth, neuronal differentiation, the establishment of the water permeability barrier in keratinocytes, and multidrug resistance in cancer cell Homo sapiens UDP + D-glucosyl-N-acylsphingosine
-
 ?
2.4.1.80 UDP-glucose + N-acylsphingosine enzyme is involved in renal growth, neuronal differentiation, the establishment of the water permeability barrier in keratinocytes, and multidrug resistance in cancer cell Homo sapiens UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
2.4.1.80 UDP-glucose + N-octanoyl sphingosine best substrate Homo sapiens UDP + D-glucosyl-N-octanoyl sphingosine
-
 ?

Subunits

EC Number Subunits Comment Organism
2.4.1.80 ?
-
 Homo sapiens
2.4.1.80 ? x * 448530, amino acid sequence determination Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
2.4.1.80 GCS
-
 Homo sapiens
2.4.1.80 GCS
-
 Canis lupus familiaris

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
2.4.1.80 37
-
 assay at Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
2.4.1.80 7.4
-
-
 Homo sapiens