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Information on EC 2.4.1.274 - glucosylceramide beta-1,4-galactosyltransferase and Organism(s) Homo sapiens
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2.4.1.274 glucosylceramide beta-1,4-galactosyltransferaseIUBMB Comments
Involved in the synthesis of several different major classes of glycosphingolipids.
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Word Map
The taxonomic range for the selected organisms is: Homo sapiens
The enzyme appears in selected viruses and cellular organisms
The enzyme appears in selected viruses and cellular organisms
Reaction Schemes
Synonyms
laccer synthase, b4galt6, beta4galt5, udp-gal:glucosylceramide beta-1,4-galactosyltransferase, lac-cer synthase, beta-1,4-galactosyltransferase v, 
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topSYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
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.
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
-
-
-
-
?
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
-
-
?
NATURAL SUBSTRATE
NATURAL 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
-
the enzyme is involved in the synthesis of several different major classes of glycosphingolipids
-
-
?
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
Mg2+
-
the enzyme is inactive without added metal ions. Maximal activity is obtained in the presence of 1.0 mM Mn2+/Mg2+ (1:1)
ACTIVATING COMPOUND
ORGANISM
UNIPROT
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]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
0.0613
recombinant enzyme, pH and temperature not specified in the publication
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
-
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
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
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
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). B4GT5_HUMAN
388
1
45119
Swiss-Prot
Secretory Pathway (Reliability: 4)
B4GT6_HUMAN
382
1
44914
Swiss-Prot
Secretory Pathway (Reliability: 4)
PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
B4GALT5 gene/protein expression is specifically increased in human colorectal cancer
APPLICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
medicine
B4GALT5 gene/protein expression is specifically increased in human colorectal cancer tumors, with a concomitant increase in its enzymatic activity, and product lactosylceramide, and increased dihydrosphingolipid metabolites. Inhibition of glycosphingolipid synthesis by the synthetic ceramide analog, D-threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol, concurrently inhibits colorectal cancer cell proliferation, as well as B4GALT5 mass and several glycosphingolipid levels
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Takizawa, M.; Nomura, T.; Wakisaka, E.; Yoshizuka, N.; Aoki, J.; Arai, H.; Inoue, K.; Hattori, M.; Matsuo, N.
cDNA cloning and expression of human lactosylceramide synthase
Biochim. Biophys. Acta
1438
301-304
1999
Homo sapiens (Q9UBX8)
Chatterjee, S.; Castiglione, E.
UDPgalactose:glucosylceramide beta1->4-galactosyltransferase activity in human proximal tubular cells from normal and familial hypercholesterolemic homozygotes
Biochim. Biophys. Acta
923
136-142
1987
Homo sapiens
Chatterjee, S.; Ghosh, N.; Khurana, S.
Purification of uridine diphosphate-galactose:glucosyl ceramide, beta1-4 galactosyltransferase from human kidney
J. Biol. Chem.
267
7148-7153
1992
Homo sapiens
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