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Information on EC 2.4.1.62 - ganglioside galactosyltransferase and Organism(s) Mus musculus and UniProt Accession Q9Z0F0
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2.4.1.62 ganglioside galactosyltransferaseIUBMB Comments
The substrate is also known as gangloside GM2, the product as gangloside GM1a
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Word Map
- 2.4.1.62
- myelin
- galactocerebroside
- galactosylceramide
- sulfatide
- cerebroside
- galactolipids
- cgalt
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beta1,3-galactosyltransferase
-
udp-glucose:ceramide
-
galcer
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myelin-specific
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2.4.1.45
- galt-4
- biotechnology
The taxonomic range for the selected organisms is: Mus musculus
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota
Synonyms
udp-galactose:ceramide galactosyltransferase, b3galt4, galt-3, gal-t2, ganglioside galactosyltransferase, beta3gal-t, beta 1,3-galactosyltransferase, gm1-synthase, udp-galactose-ceramide galactosyltransferase, 
more
topSYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
galactosyltransferase, uridine diphosphogalactose-ceramide
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GM1-synthase
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UDP galactose-LAC Tet-ceramide alpha-galactosyltransferase
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UDP-galactose-ceramide galactosyltransferase
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UDP-galactose-GM2 galactosyltransferase
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UDP-galactose-GM2 ganglioside galactosyltransferase
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UDP-galactose:N-acetylgalactosaminyl-(N-acetylneuraminyl) galactosyl-glucosyl-ceramide galactosyltransferase
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uridine diphosphate D-galactose:glycolipid galactosyltransferase
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uridine diphosphogalactose-ceramide galactosyltransferase
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uridine diphosphogalactose-GM2 galactosyltransferase
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
hexosyl group transfer
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-
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SYSTEMATIC NAME
IUBMB Comments
UDP-alpha-D-galactose:N-acetyl-beta-D-galactosaminyl-(1->4)-[alpha-N-acetylneuraminyl-(2->3)]-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide 3-beta-D-galactosyltransferase
The substrate is also known as gangloside GM2, the product as gangloside GM1a
CAS REGISTRY NUMBER
COMMENTARY
37217-28-0
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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 + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
UDPgalactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
additional information
?
-
-
mice heterozygous for a non-functional allele of UDPgalactose:ceramide-galactosyltransferase show decreased synthesis of galactosylceramide and sulfatide, which should reduce sulfatide storage and improve pathology in arylsulfatase A-deficient mice. The phenotypic and pathologic improvements in ASA-/- CGT+/- mice are significant but overall minor
-
-
?
UDP-galactose + ganglioside GA2
UDP + ganglioside GA1
i.e. Cer-Glc-Gal-GalNAc
i.e. Cer-Glc-Gal-GalNAc-Gal
?
UDP-galactose + ganglioside GA2
UDP + ganglioside GA1
i.e. Cer-Glc-Gal-GalNAc
i.e. Cer-Glc-Gal-GalNAc-Gal
?
UDP-galactose + GD2
UDP + Gd1b
i.e. Cer-Glc-Gal-(GalNAc)(NeuNAc2)
i.e. Cer-Glc-Gal-(GalNAc-Gal)(NeuNAc2)
?
UDP-galactose + GD2
UDP + Gd1b
i.e. Cer-Glc-Gal-(GalNAc)(NeuNAc2)
i.e. Cer-Glc-Gal-(GalNAc-Gal)(NeuNAc2)
?
UDP-galactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
-
i.e. GM1
?
UDP-galactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
-
i.e. GM1
?
UDPgalactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
involved in biosynthesis of gangliosides
i.e. GM1
?
UDPgalactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
involved in biosynthesis of gangliosides
i.e. GM1
?
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
UDPgalactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
additional information
?
-
-
mice heterozygous for a non-functional allele of UDPgalactose:ceramide-galactosyltransferase show decreased synthesis of galactosylceramide and sulfatide, which should reduce sulfatide storage and improve pathology in arylsulfatase A-deficient mice. The phenotypic and pathologic improvements in ASA-/- CGT+/- mice are significant but overall minor
-
-
?
UDP-galactose + ganglioside GA2
UDP + ganglioside GA1
i.e. Cer-Glc-Gal-GalNAc
i.e. Cer-Glc-Gal-GalNAc-Gal
?
UDP-galactose + ganglioside GA2
UDP + ganglioside GA1
i.e. Cer-Glc-Gal-GalNAc
i.e. Cer-Glc-Gal-GalNAc-Gal
?
UDP-galactose + GD2
UDP + Gd1b
i.e. Cer-Glc-Gal-(GalNAc)(NeuNAc2)
i.e. Cer-Glc-Gal-(GalNAc-Gal)(NeuNAc2)
?
UDP-galactose + GD2
UDP + Gd1b
i.e. Cer-Glc-Gal-(GalNAc)(NeuNAc2)
i.e. Cer-Glc-Gal-(GalNAc-Gal)(NeuNAc2)
?
UDPgalactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
involved in biosynthesis of gangliosides
i.e. GM1
?
UDPgalactose + N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
UDP + D-galactosyl-1,3-beta-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosyl-N-acylsphingosine
involved in biosynthesis of gangliosides
i.e. GM1
?
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
PP55B
-
i.e. isopropylidene derivative of 5'-O-[[(2-decanoylamino-3-phenylpropylloxycarbonyl)amino]sulfonyl]uridine, 21% inhibition at 0.2 mM
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.023
N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
0.023
N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
recombinant fusion protein
0.023
N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-1,4-beta-D-glucosyl-N-acylsphingosine
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recombinant fusion protein
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
0.00000005
recombinant enzyme, crude cell homogenate of transfected NIH 3T3 cells
0.000000051
recombinant enzyme, crude cell homogenate of transfected CHO-K1 cells
0.00000007
recombinant enzyme, crude cell homogenate of transfected CHO-K1 cells
ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction
-
Cgt deficient mice, no accumulation of galactolipids, increased ceramide levels
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). B3GT4_MOUSE
371
1
41236
Swiss-Prot
Secretory Pathway (Reliability: 1)
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
43000
additional information
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
glycoprotein
additional information
glycoprotein
inhibition of glycosylation by tunicamycin leads to an inactive enzyme and enzyme targeting to the endoplasmatic reticulum instead of the Golgi apparatus
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
additional information
point mutation at the glycosylation site results in loss of activity and enzyme targeting to the endoplasmic reticulum instead of the Golgi apparatus
additional information
-
point mutation at the glycosylation site results in loss of activity and enzyme targeting to the endoplasmic reticulum instead of the Golgi apparatus
additional information
-
Cgt-/-, Cgt deficient mice, no accumulation of galactolipids, increased ceramide levels
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
cloning and DNA sequence determination and analysis, expression in CHO-K1 and NIH-3T3 cells as fusion protein tagged with a nonapeptide from influenza virus hemagglutinin, amino acid sequence determination
expression in CHO-K1 cells as fusion protein tagged with a nonapeptide from influenza virus hemagglutinin
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Daniotti, J.L.; Martina, J.A.; Zurita, A.R.; Maccioni, H.J.
Mouse beta 1,3-galactosyltransferase (GA1/GM1/GD1b synthase): protein characterization, tissue expression, and developmental regulation in neural retina
J. Neurosci. Res.
58
318-327
1999
Mus musculus (Q9Z0F0), Mus musculus
Martina, J.A.; Daniotti, J.L.; Maccioni, H.J.
GM1 synthase depends on N-glycosylation for enzyme activity and trafficking to the Golgi complex
Neurochem. Res.
25
725-731
2000
Mus musculus (Q9Z0F0), Mus musculus
Paul, P.; Lutz, T.M.; Osborn, C.; Kyosseva, S.; Elbein, A.D.; Towbin, H.; Radominska, A.; Drake, R.R.
Synthesis and characterization of a new class of inhibitors of membrane-associated UDP-glycosyltransferases
J. Biol. Chem.
268
12933-12938
1993
Mus musculus
Franken, S.; Wittke, D.; Mansson, J.E.; DHooge, R.; De Deyn, P.P.; Luellmann-Rauch, R.; Matzner, U.; Gieselmann, V.
Modest phenotypic improvements in ASA-deficient mice with only one UDP-galactose:ceramide-galactosyltransferase gene
Lipids Health Dis.
5
21
2006
Mus musculus
Saadat, L.; Dupree, J.L.; Kilkus, J.; Han, X.; Traka, M.; Proia, R.L.; Dawson, G.; Popko, B.
Absence of oligodendroglial glucosylceramide synthesis does not result in CNS myelin abnormalities or alter the dysmyelinating phenotype of CGT-deficient mice
Glia
58
391-398
2010
Mus musculus
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