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EC Tree
IUBMB Comments The substrate is also known as gangloside GM2, the product as gangloside GM1a
The taxonomic range for the selected organisms is: Homo sapiens The expected taxonomic range for this enzyme is: Bacteria, Eukaryota
Synonyms
udp-galactose:ceramide galactosyltransferase, b3galt4, galt-3, gal-t2, beta3gal-t, ganglioside galactosyltransferase, beta 1,3-galactosyltransferase, gm1-synthase, udp-galactose-ceramide galactosyltransferase,
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UDP-galactose:beta-N-acetylglucosamine beta-1,3-galactosyltransferase
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ceramide galactosyltransferase UGT8
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galactosyltransferase, uridine diphosphogalactose-ceramide
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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:beta-N-acetylglucosamine beta-1,3-galactosyltransferase
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UDP-galactose:ceramide 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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hexosyl group transfer
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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
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UDP-galactose + ganglioside GA2
UDP + ganglioside GA1
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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
donor and acceptor specificity, overview
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UDP-galactose + N-acetyl-Gal-glycosylphosphatidylinositol
UDP + ?
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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
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key enzyme in the biosynthesis of the oligodendrocyte- and myelin-specific cerebrosides
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additional information
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GM1 ganglioside synthase B3GALT4 additionally functions in transferring galactose to the N-acetylgalactosamine side-chain of GPI. B3GALT4 requires lactosylceramide for the efficient GPI side-chain galactosylation
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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
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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
donor and acceptor specificity, overview
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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
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key enzyme in the biosynthesis of the oligodendrocyte- and myelin-specific cerebrosides
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lactosylceramide
presence of lactosylceramide is required for B3GALT4 to transfer galalctose to glycosylphosphatidylinositol
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Adenoma
Ceramide galactosyltransferase (UGT8) as a molecular marker of canine mammary tumor malignancy.
Alzheimer Disease
DNA Hypomethylation in Blood Links B3GALT4 and ZADH2 to Alzheimer's Disease.
Breast Neoplasms
Ceramide galactosyltransferase (UGT8) is a molecular marker of breast cancer malignancy and lung metastases.
Breast Neoplasms
Galactosylceramide affects tumorigenic and metastatic properties of breast cancer cells as an anti-apoptotic molecule.
Breast Neoplasms
Molecular basis of incomplete O-glycan synthesis in MCF-7 breast cancer cells: putative role of MUC6 in Tn antigen expression.
Carcinoma
Benzyl-N-acetyl-alpha-D-galactosaminide inhibits the sialylation and the secretion of mucins by a mucin secreting HT-29 cell subpopulation.
Carcinoma
Ceramide galactosyltransferase (UGT8) as a molecular marker of canine mammary tumor malignancy.
Cholestasis
Changes in GM1 ganglioside content and localization in cholestatic rat liver.
Colonic Neoplasms
beta 1,3-Galactosyltransferase beta 3Gal-T5 acts on the GlcNAcbeta 1-->3Galbeta 1-->4GlcNAcbeta 1-->R sugar chains of carcinoembryonic antigen and other N-linked glycoproteins and is down-regulated in colon adenocarcinomas.
Colorectal Neoplasms
Clinical correlation of B7-H3 and B3GALT4 with the prognosis of colorectal cancer.
Demyelinating Diseases
Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase.
Leukodystrophy, Globoid Cell
Glycosylceramide synthesis in the developing spinal cord and kidney of the twitcher mouse, an enzymatically authentic model of human Krabbe disease.
Neoplasm Metastasis
Ceramide galactosyltransferase (UGT8) is a molecular marker of breast cancer malignancy and lung metastases.
Neoplasm Metastasis
Galactosylceramide affects tumorigenic and metastatic properties of breast cancer cells as an anti-apoptotic molecule.
Neoplasms
Benzyl-N-acetyl-alpha-D-galactosaminide inhibits the sialylation and the secretion of mucins by a mucin secreting HT-29 cell subpopulation.
Neoplasms
Galactosylceramide affects tumorigenic and metastatic properties of breast cancer cells as an anti-apoptotic molecule.
Pancreatic Neoplasms
Association between expression levels of CA 19-9 and N-acetylglucosamine-beta;1,3-galactosyltransferase 5 gene in human pancreatic cancer tissue.
Paralysis
Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase.
Sarcoma
Absence of a specific ganglioside galactosyltransferase in mouse cells transformed by murine sarcoma virus.
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additional information
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isoform B3GALT4
SwissProt
brenda
isoform beta3Gal-T1
SwissProt
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isoform beta3Gal-T3
SwissProt
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additional information
widely distributed
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additional information
widely distributed
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additional information
widely distributed
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additional information
widely distributed
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additional information
widely distributed
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additional information
widely distributed
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physiological function
glycosylphosphatidylinositol bearing non-galactosylated GalNAc side-chain increases from 70% to 81% after KO of B3GALT4, however, 18% of the GPI still have one hexose and a GalNAc side-chain
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B3GT4_HUMAN
378
1
41537
Swiss-Prot
Secretory Pathway (Reliability: 1 )
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additional information
amino acid sequence alignment
additional information
amino acid sequence alignment
additional information
amino acid sequence alignment
additional information
amino acid sequence alignment
additional information
amino acid sequence alignment
additional information
amino acid sequence alignment
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glycoprotein
glycosylation sites, overview
glycoprotein
glycosylation sites, overview
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D175A
overexpression of the mutant and -D291A does not rescue galactosylation deficiency of a knockout strain
D176A
overexpression of the mutant and -D291A does not rescue galactosylation deficiency of a knockout strain
D177A
overexpression of the mutant and -D291A does not rescue galactosylation deficiency of a knockout strain
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amino acid sequence determination, expression in Sf9 insect cells via baculovirus transfection
cloning and DNA sequencing, amino acid sequence determination, expression in Sf9 insect cells via baculovirus transfection
cloning and DNA sequencing, amino acid sequence determination, expression in Sf9 insect cells via baculovirus transfection
cloning from human cDNA library, promotor identification via primer extension analysis, deletion and cloning of several 5'-end constructs, transient expression in human cell lines derived from oligodendroglioma and neuroblastoma LAN-5, promotor activity is cell type-specific, identification of regulatory elements
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molecular marker of breast cancer malignancy and lung metastases, significant index of tumour aggressiveness and a potential marker for the prognostic evaluation of lung metastases in breast cancer
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Tencomnao, T.; Yu, R.K.; Kapitonov, D.
Characterization of the human UDP-galactose:ceramide galactosyltransferase gene promoter
Biochim. Biophys. Acta
1517
416-423
2001
Homo sapiens
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Amado, M.; Almeida, R.; Carneiro, F.; Levery, S.B.; Holmes, E.H.; Nomoto, M.; Hollingsworth, M.A.; Hassan, H.; Schwientek, T.; Nielsen, P.A.; Bennett, E.P.; Clausen, H.
A family of human beta3-galactosyltransferases. Characterization of four members of a UDP-galactose:beta-N-acetyl-glucosamine/beta-acetyl-galactosamine beta-1,3-galactosyltransferase family
J. Biol. Chem.
273
12770-12778
1998
Homo sapiens (O43825), Homo sapiens (O75752), Homo sapiens (O96024), Drosophila melanogaster (Q24157)
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Dziegiel, P.; Owczarek, T.; Plazuk, E.; Gomulkiewicz, A.; Majchrzak, M.; Podhorska-Okolow, M.; Driouch, K.; Lidereau, R.; Ugorski, M.
Ceramide galactosyltransferase (UGT8) is a molecular marker of breast cancer malignancy and lung metastases
Br. J. Cancer
103
524-531
2010
Homo sapiens
brenda
Wang, Y.; Maeda, Y.; Liu, Y.S.; Takada, Y.; Ninomiya, A.; Hirata, T.; Fujita, M.; Murakami, Y.; Kinoshita, T.
Cross-talks of glycosylphosphatidylinositol biosynthesis with glycosphingolipid biosynthesis and ER-associated degradation
Nat. Commun.
11
860
2020
Homo sapiens (O96024)
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