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Enzyme Nomenclature
Information on EC 2.4.1.274 - glucosylceramide beta-1,4-galactosyltransferase
for references in articles please use BRENDA:EC2.4.1.274
Word Map on EC 2.4.1.274
- 2.4.1.274
- lactosylceramide
-
glycosphingolipids
- gm3
- beta-1,4-galts
-
d-pdmp
-
galt-2
-
galcers
- d-threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol
- medicine
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The enzyme appears in viruses and cellular organisms
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EC NUMBER 
COMMENTARY 
2.4.1.274
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RECOMMENDED NAME
GeneOntology No.
glucosylceramide beta-1,4-galactosyltransferase
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
UDP-alpha-D-galactose + beta-D-glucosyl-(1<->1)-ceramide = UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
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PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
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SYSTEMATIC NAME
IUBMB Comments
UDP-galactose:beta-D-glucosyl-(1<->1)-ceramide beta-1,4-galactosyltransferase
Involved in the synthesis of several different major classes of glycosphingolipids.
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
physiological function
physiological function
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elevated glucosylceramide beta-1,4-galactosyltransferase activity may contribute to the storage of lactosylceramide urinary proximal tubular cells from familial hypercholesterolemic patients
physiological function
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B4galt6 KO mice are born and grow up apparently normal. LacCer synthase activity and the composition of acidic glycosphingolipids in the brain are almost equivalent or minimally different between wild-type and KO mice. B4GalT6 does not act as a major beta4GalT on the modification of glycoproteins
physiological function
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the amounts of Lac-Cer and ganglioside GM3 are significantly less in a stable clone E5 whose beta4GalT5 gene expression level is reduced to 35% than in a control clone C1 by transfection of its antisense cDNA. The average tumor size and average number of colonies formed with clone E5 are decreased to 44 and 49%, respectively, of those formed with clone C1. The numbers and sizes of colonies formed in the soft agarose gels, and the volumes of tumors formed in athymic mice with fibroblasts from wild type, heterozygous and homozygous beta4GalT5-knockout mouse embryos upon transformation with the polyoma virus oncogene correlate with the beta4GalT5 gene dosage
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
UDP-galactose + GlcNAc-beta-(1->3)-Gal-beta-(1->4)-Glc-beta-(1<->1)-Cer
?
-
-
-
-
?
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
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-
-
-
?
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
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the enzyme is involved in the synthesis of several different major classes of glycosphingolipids
-
-
?
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
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-
-
-
?
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NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
-
the enzyme is involved in the synthesis of several different major classes of glycosphingolipids
-
-
?
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
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-
-
-
?
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Ca2+
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the enzyme is activated by Mn2+ and to a lesser extent by Mg2+ and Ca2+
Mg2+
Mn2+
Mg2+
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the enzyme is inactive without added metal ions. Maximal activity is obtained in the presence of 1.0 mM Mn2+/Mg2+ (1:1)
Mg2+
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the enzyme is activated by Mn2+ and to a lesser extent by Mg2+ and Ca2+
Mn2+
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the enzyme is activated by Mn2+ and to a lesser extent by Mg2+ and Ca2+
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
Cutscum
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maximal activity in a detergent mixture, Triton X-100/Cutscum (1:2, v/v), 0.1 mg/ml
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Triton X-100
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maximal activity in a detergent mixture, Triton X-100/Cutscum (1:2, v/v), 0.1 mg/ml
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
0.0613
recombinant enzyme, pH and temperature not specified in the publication
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
37
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pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
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isoform beta4GalT5 works predominantly in mouse embryonic fibroblasts
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from normal and familial hypercholesterolemic homozygotes. Urinary proximal tubular cells from familial hypercholesterolemic homozygous patients contain glucosylceramide beta-1,4-galactosyltransferase activity 35% higher as compared to control cells
additional information
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expression levels of B4galt6 gene is almost equivalent between male and female mice
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male mice show much higher activity of LacCer synthesis than female mice in the kidney. Expression levels of the B4galt5 gene correlate with the LacCer synthase activity in the kidney
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
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MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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SUBUNITS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
glycoprotein
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contains about 15% N-linked carbohydrate. The polypeptide contains eight putative glycosylation sites
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Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
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Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
expression in Sf9 cells
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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
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APPLICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
medicine
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the amounts of Lac-Cer synthesized by isoform beta4GalT5 correlate with the tumorigenic potentials of malignantly transformed cells
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LINKS TO OTHER DATABASES (specific for EC-Number 2.4.1.274)
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