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Information on EC 2.4.1.134 - galactosylxylosylprotein 3-beta-galactosyltransferase and Organism(s) Homo sapiens

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IUBMB Comments
Involved in the biosynthesis of the linkage region of glycosaminoglycan chains as part of proteoglycan biosynthesis (chondroitin, dermatan and heparan sulfates). Requires Mn2+.
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This record set is specific for:
Homo sapiens
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The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Synonyms
galt2, dbeta3galtii, beta3galt i, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
beta3GalT I
-
beta3GalT II
-
-
beta3GalT IV
-
-
beta3GalT V
-
-
beta3GalT VI
-
beta3GalT6
-
galactosyltransferase II
galactosyltransferase, uridine diphosphogalactose-galactosylxylose
-
-
-
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galactosyltransferase-2
-
-
GalT2
-
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UDPgalactose:4-beta-D-galactosyl-O-beta-D-xylosylprotein 3-beta-D-galactosyltransferase
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
hexosyl group transfer
-
-
-
-
SYSTEMATIC NAME
IUBMB Comments
UDP-galactose:4-beta-D-galactosyl-O-beta-D-xylosylprotein 3-beta-D-galactosyltransferase
Involved in the biosynthesis of the linkage region of glycosaminoglycan chains as part of proteoglycan biosynthesis (chondroitin, dermatan and heparan sulfates). Requires Mn2+.
CAS REGISTRY NUMBER
COMMENTARY hide
56626-19-8
-
56626-21-2
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
UDP-alpha-D-galactose + 4-beta-D-galactosyl-O-beta-D-xylosyl-[protein]
UDP + 3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosyl-[protein]
show the reaction diagram
-
-
-
?
UDP-alpha-D-galactose + 4-methylumbelliferyl-beta-D-xylopyranoside
UDP + ?
show the reaction diagram
-
-
-
?
UDP-galactose + beta-D-galactosyl-1,3-beta-galactosyl-O-naphthalenemethanol
UDP + beta-D-galactosyl-1,3-beta-D-galactosyl-1,3-beta-galactosyl-O-naphthalenemethanol
show the reaction diagram
-
-
-
-
?
UDP-galactose + beta-D-galactosyl-1,3-N-acetyl-alpha-D-glucosaminyl-O-naphthalenemethanol
UDP + beta-D-galactosyl-1,3-beta-D-galactosyl-1,3-N-acetyl-alpha-D-glucosaminyl-O-naphthalenemethanol
show the reaction diagram
-
-
-
-
?
UDP-galactose + beta-D-galactosyl-1,4-beta-D-xylosyl-O-benzyl
UDP + beta-D-galactosyl-1,3-beta-D-galactosyl-1,4-beta-D-xylosyl-O-benzyl
show the reaction diagram
-
-
-
-
?
UDP-galactose + beta-D-galactosyl-1,4-N-acetyl-beta-D-glucosaminyl-O-naphthalenemethanol
UDP + beta-D-galactosyl-1,3-beta-D-galactosyl-1,4-N-acetyl-beta-D-glucosaminyl-O-naphthalenemethanol
show the reaction diagram
-
-
-
-
?
UDP-galactose + beta-D-galactosyl-O-naphthalenemethanol
UDP + beta-D-galactosyl-1,3-beta-D-galactosyl-O-naphthalenemethanol
show the reaction diagram
-
-
-
-
?
additional information
?
-
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
UDP-alpha-D-galactose + 4-beta-D-galactosyl-O-beta-D-xylosyl-[protein]
UDP + 3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosyl-[protein]
show the reaction diagram
-
-
-
?
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
1
-
pmol/h/ml medium, beta-D-galactosyl-1,3-beta-D-galactosyl-O-naphthalenemethanol as substrate
18
-
pmol/h/ml medium, beta-D-galactosyl-O-naphthalenemethanol as substrate
4
-
pmol/h/ml medium, beta-D-galactosyl-1,4-N-acetyl-beta-D-glucosaminyl-O-naphthalenemethanol as substrate
6
-
pmol/h/ml medium, beta-D-galactosyl-1,3-N-acetyl-alpha-D-glucosaminyl-O-naphthalenemethanol as substrate
84
-
pmol/h/ml medium, beta-D-galactosyl-1,4-beta-xylosyl-O-benzyl as substrate
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
metabolism
the enzyme catalyzes a step in glycosaminoglycan synthesis, pathway overview
physiological function
proteoglycans are important components of cell plasma membranes and extracellular matrices of connective tissues. They consist of glycosaminoglycan chains attached to a core protein via a tetrasaccharide linkage, whereby addition of the third residue is catalyzed by galactosyltransferase II, encoded by gene B3GALT6. A crucial role for b3GalT6 in multiple major developmental and pathophysiological processes
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
B3GT6_HUMAN
329
1
37138
Swiss-Prot
Secretory Pathway (Reliability: 3)
B3GT1_HUMAN
326
1
37993
Swiss-Prot
other Location (Reliability: 3)
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
A108G
naturally occuring enzyme mutation
D207H
naturally occuring enzyme mutation
G217S
naturally occuring enzyme mutation
additional information
homozygosity mapping and candidate gene sequence analysis in three independent families identified biallelic gene B3GALT6 mutations, including homozygous missense mutations in family 1 (c.619G>C [p.Asp207His]) and family 3 (c.649G>A [p.Gly217Ser]) and compound heterozygous mutations in family 2 (c.323_344del [p.Ala108Glyfs*163], c.619G>C [p.Asp207His]), phenotypes overview
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
gene B3GALT6, homozygosity mapping and candidate gene sequence analysis in three independent families, genotyping
transient expression as a fusion protein with protein A in chinese hamster ovary cells COS-1
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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
there is no significant effect of Sry-type high-mobility-group box 9 transduction on the gene expression of galactosyltransferase-2
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Bai, X.; Zhou, D.; Brown, J.R.; Crawford, B.E.; Hennet, T.; Esko, J.D.
Biosynthesis of the linkage region of glycosaminoglycans. Cloning and activity of galactosyltransferase II, the sixth member of the beta1,3-galactosyltransferase family (beta3GalT6)
J. Biol. Chem.
276
48189-48195
2001
Homo sapiens, Mus musculus
Manually annotated by BRENDA team
Patel, R.Y.; Balaji, P.V.
Fold-recognition and comparative modeling of human beta3GalTI, II, IV, V and VI and beta 3GalNAcT I: Prediction of residues conferring acceptor substrate specificity
J. Mol. Graph. Model.
26
255-268
2007
Homo sapiens, Homo sapiens (Q96L58), Homo sapiens (Q9Y5Z6)
Manually annotated by BRENDA team
Tew, S.R.; Pothacharoen, P.; Katopodi, T.; Hardingham, T.E.
SOX9 transduction increases chondroitin sulfate synthesis in cultured human articular chondrocytes without altering glycosyltransferase and sulfotransferase transcription
Biochem. J.
414
231-236
2008
Homo sapiens
Manually annotated by BRENDA team
Malfait,F.; Kariminejad, A.; Van Damme,T.; Gauche, C.; Syx, D.; Merhi-Soussi, F.; Gulberti, S.; Symoens, S.; Vanhauwaert, S.; Willaert, A.; Bozorgmehr, B.; Kariminejad, M.H.; Ebrahimiadib, N.; Hausser, I.; Huysseune, A.; Fournel-Gigleux, S.; De Paepe, A.
Defective initiation of glycosaminoglycan synthesis due to B3GALT6 mutations causes a pleiotropic Ehlers-Danlos-Syndrome-like connective tissue disorder
Am. J. Hum. Genet.
92
935-945
2013
Homo sapiens (Q96L58)
Manually annotated by BRENDA team