Information on EC 2.4.1.274 - glucosylceramide beta-1,4-galactosyltransferase

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The expected taxonomic range for this enzyme is: Euarchontoglires

EC NUMBER
COMMENTARY
2.4.1.274
-
RECOMMENDED NAME
GeneOntology No.
glucosylceramide beta-1,4-galactosyltransferase
-
REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
UDP-alpha-D-galactose + beta-D-glucosyl-(1<->1)-ceramide = UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
show the reaction diagram
-
-
-
-
PATHWAY
KEGG Link
MetaCyc Link
Metabolic pathways
-
Sphingolipid metabolism
-
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.
SYNONYMS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
LacCer synthase
Q9UBX8
-
LacCer synthase
-
-
lactosylceramide synthase
Q9UBX8
-
lactosylceramide synthase
-
-
UDP-Ga1:glucosylceramide beta1->4galactosyltransferase
-
-
UDP-Gal:glucosylceramide beta-1,4-galactosyltransferase
-
-
UDP-galactose:GlcCer, beta1-4 galactosyltransferase
-
-
UDPgalactose:glucosylceramide beta1->4-galactosyltransferase
-
-
ORGANISM
COMMENTARY
LITERATURE
SEQUENCE CODE
SEQUENCE DB
SOURCE
-
SwissProt
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
physiological function
-
elevated glucosylceramide beta-1,4-galactosyltransferase activity may contribute to the storage of lactosylceramide urinary proximal tubular cells from familial hypercholesterolemic patients
SUBSTRATE
PRODUCT                      
REACTION DIAGRAM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
(Substrate)
LITERATURE
(Substrate)
COMMENTARY
(Product)
LITERATURE
(Product)
Reversibility
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
show the reaction diagram
-
-
-
-
?
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
show the reaction diagram
-
-
-
-
?
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
show the reaction diagram
-
the enzyme is involved in the synthesis of several different major classes of glycosphingolipids
-
-
?
UDP-galactose + ceramide
?
show the reaction diagram
-
-
-
-
?
UDP-galactose + galactosylceramide
?
show the reaction diagram
-
-
-
-
?
UDP-galactose + GlcNAc-beta-(1->3)-Gal-beta-(1->4)-Glc-beta-(1<->1)-Cer
?
show the reaction diagram
-
-
-
-
?
UDP-galactose + lactosylceramide
?
show the reaction diagram
-
-
-
-
?
NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
(Substrate)
LITERATURE
(Substrate)
COMMENTARY
(Product)
LITERATURE
(Product)
REVERSIBILITY
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
show the reaction diagram
-
-
-
-
?
UDP-galactose + beta-D-glucosyl-(1<->1)-ceramide
UDP + beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide
show the reaction diagram
-
the enzyme is involved in the synthesis of several different major classes of glycosphingolipids
-
-
?
METALS and IONS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
Ca2+
-
the enzyme is activated by Mn2+ and to a lesser extent by Mg2+ and Ca2+
Mg2+
-
the enzyme is inactive without added metal ions. Maximal activity is obtained in the presence of 1.0 mM Mn2+/Mg2+ (1:1)
Mg2+
-
the enzyme is activated by Mn2+ and to a lesser extent by Mg2+ and Ca2+
Mn2+
-
required
Mn2+
-
optimum activity is obtained in the presence of 1.0 mM Mn2+/Mg2+ (1:1)
Mn2+
-
the enzyme is activated by Mn2+ and to a lesser extent by Mg2+ and Ca2+
ACTIVATING COMPOUND
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
Cutscum
-
maximal activity in a detergent mixture, Triton X-100/Cutscum (1:2, v/v), 0.1 mg/ml
-
Triton X-100
-
maximal activity in a detergent mixture, Triton X-100/Cutscum (1:2, v/v), 0.1 mg/ml
KM VALUE [mM]
KM VALUE [mM] Maximum
SUBSTRATE
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
IMAGE
0.003
-
beta-D-glucosyl-(1<->1)-ceramide
-
pH 6.8, 37C
0.0237
-
beta-D-glucosyl-(1<->1)-ceramide
-
pH 7.4, 37C
0.11
-
beta-D-glucosyl-(1<->1)-ceramide
-
pH 7.0, 37C
0.0005
-
UDP-galactose
-
pH 6.8, 37C
0.0806
-
UDP-galactose
-
pH 7.4, 37C
0.52
-
UDP-galactose
-
pH 7.0, 37C
SPECIFIC ACTIVITY [µmol/min/mg]
SPECIFIC ACTIVITY MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
0.0613
-
Q9UBX8
recombinant enzyme, pH and temperature not specified in the publication
pH OPTIMUM
pH MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
6.8
-
-
assay at
7.2
-
-
cacodylate buffer
7.4
-
-
assay at
TEMPERATURE OPTIMUM
TEMPERATURE OPTIMUM MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
37
-
-
assay at
37
-
-
assay at
37
-
-
assay at
pI VALUE
pI VALUE MAXIMUM
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
4.55
-
-
isoelectric focusing
SOURCE TISSUE
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
SOURCE
Q9UBX8
high expression
Manually annotated by BRENDA team
Q9UBX8
high expression
Manually annotated by BRENDA team
-
high activity
Manually annotated by BRENDA team
Q9UBX8
low axpression
Manually annotated by BRENDA team
Q9UBX8
low axpression
Manually annotated by BRENDA team
Q9UBX8
low axpression
Manually annotated by BRENDA team
-
low activity
Manually annotated by BRENDA team
Q9UBX8
low axpression
Manually annotated by BRENDA team
-
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
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
SUBUNITS
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
?
-
x * 44776, calculated from sequence; x * 61000, SDS-PAGE
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT ACCESSION NO.
COMMENTARY
LITERATURE
glycoprotein
-
contains about 15% N-linked carbohydrate. The polypeptide contains eight putative glycosylation sites
Purification/COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
Cloned/COMMENTARY
ORGANISM
UNIPROT ACCESSION NO.
LITERATURE
expression in Sf9 cells
Q9UBX8
expression in Sf9 cells
-