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Literature summary for 2.4.1.80 extracted from

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

Activating Compound

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

Application

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

Cloned(Commentary)

Cloned (Comment) Organism
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

Inhibitors Comment Organism Structure
CDP-glucose slight inhibition Homo sapiens
Cu2+
-
Homo sapiens
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
D-threo-1-phenyl-2-(decanoylamino)-3-morpholino-1-propanol-HCl
-
Homo sapiens
Fe2+
-
Homo sapiens
Fe3+
-
Homo sapiens
additional information induction of enzyme by inhibitors in presence of higher ceramide levels Canis lupus familiaris
additional information induction of enzyme by inhibitors in presence of higher ceramide levels; no inhibition by conduritol B epoxide Homo sapiens
N-butyldeoxynojirimycin
-
Canis lupus familiaris
N-butyldeoxynojirimycin nonspecific inhibitor Homo sapiens
Tris 58% loss of activity at 0.2 M Homo sapiens
Zn2+
-
Homo sapiens

Metals/Ions

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

Molecular Weight [Da]

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

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
UDP-glucose + N-acylsphingosine Canis lupus familiaris
-
UDP + D-glucosyl-N-acylsphingosine
-
?
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
-
?
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

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

Source Tissue

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

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information octanoyl dihydrosphingosine, decanoyl sphingosine, and stearoyl sphingosine are poor substrates Homo sapiens ?
-
?
UDP-glucose + N-acylsphingosine
-
Canis lupus familiaris UDP + D-glucosyl-N-acylsphingosine
-
?
UDP-glucose + N-acylsphingosine acceptor substrate specificity, overview Homo sapiens UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
UDP-glucose + N-acylsphingosine i.e. ceramide Homo sapiens UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
UDP-glucose + N-acylsphingosine i.e. ceramide Canis lupus familiaris UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
UDP-glucose + N-acylsphingosine overview: short-chain ceramide substrates Homo sapiens UDP + D-glucosyl-N-acylsphingosine i.e. glucosylceramide ?
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
-
?
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 ?
UDP-glucose + N-octanoyl sphingosine best substrate Homo sapiens UDP + D-glucosyl-N-octanoyl sphingosine
-
?

Subunits

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

Synonyms

Synonyms Comment Organism
GCS
-
Homo sapiens
GCS
-
Canis lupus familiaris

Temperature Optimum [°C]

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

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.4
-
-
Homo sapiens