Information on EC 2.4.1.174 - glucuronylgalactosylproteoglycan 4-beta-N-acetylgalactosaminyltransferase and Organism(s) Homo sapiens

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Homo sapiens


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The enzyme appears in selected viruses and cellular organisms

EC NUMBER
COMMENTARY hide
2.4.1.174
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RECOMMENDED NAME
GeneOntology No.
glucuronylgalactosylproteoglycan 4-beta-N-acetylgalactosaminyltransferase
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
hexosyl group transfer
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PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
chondroitin biosynthesis
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Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate
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Metabolic pathways
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SYSTEMATIC NAME
IUBMB Comments
UDP-N-acetyl-D-galactosamine:D-glucuronyl-(1->3)-beta-D-galactosyl-proteoglycan 4-beta-N-acetylgalactosaminyltransferase
Requires Mn2+. Involved in the biosynthesis of chondroitin sulfate. Key enzyme activity for the initiation of chondroitin and dermatan sulfates, transferring GalNAc to the GlcA-Gal-Gal-Xyl-Ser core.
CAS REGISTRY NUMBER
COMMENTARY hide
96189-39-8
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ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
physiological function
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
UDP-N-acetyl-D-galactosamine + beta-D-glucuronyl-(1->3)-D-galactosyl-proteoglycan
UDP + N-acetyl-D-galactosaminyl-(1->4)-beta-D-glucuronyl-(1->3)-beta-D-galactosylproteoglycan
show the reaction diagram
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?
UDP-N-acetyl-D-galactosamine + beta-D-glucuronyl-1,3-beta-D-galactosyl-1,3-beta-D-galactosyl-1,4-beta-D-xylosyl-1-O-(Gly)Ser-(Gly-Glu)
UDP + N-acetyl-D-galactosamine-1,4-beta-D-glucuronyl-1,3-beta-D-galactosyl-1,3-beta-D-galactosyl-1,4-beta-D-xylosyl-1-O-(Gly)Ser-(Gly-Glu)
show the reaction diagram
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?
UDP-N-acetyl-D-galactosamine + beta-D-glucuronyl-1,3-beta-D-galactosyl-1,3-beta-D-galactosyl-1,4-beta-D-xylosyl-1-O-Ser
UDP + N-acetyl-D-galactosamine-1,4-beta-D-glucuronyl-1,3-beta-D-galactosyl-1,3-beta-D-galactosyl-1,4-beta-D-xylosyl-1-O-Ser
show the reaction diagram
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?
UDP-N-acetyl-D-galactosamine + beta-D-glucuronyl-1,3-beta-D-galactosyl-1,3-beta-D-galactosyl-1,4-beta-D-xylosyl-1-O-Ser-peptide
UDP + N-acetyl-D-galactosamine-1,4-beta-D-glucuronyl-1,3-beta-D-galactosyl-1,3-beta-D-galactosyl-1,4-beta-D-xylosyl-1-O-Ser-peptide
show the reaction diagram
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?
UDP-N-acetyl-D-galactosamine + beta-D-glucuronyl-1,3-beta-D-galactosyl-1,3-beta-D-galactosyl-beta-D-xylose-O-methoxyphenyl
UDP + N-acetyl-D-galactosamine-1,4-beta-D-glucuronyl-1,3-beta-D-galactosyl-1,3-beta-D-galactosyl-beta-D-xylose-O-methoxyphenyl
show the reaction diagram
UDP-N-acetyl-D-galactosamine + beta-D-glucuronyl-1,3-beta-D-galactosyl-1-O-C2H4-NH-benzyloxycarbonyl
UDP + N-acetyl-D-galactosamine-1,4-beta-D-glucuronyl-1,3-beta-D-galactosyl-1-O-C2H4-NH-benzyloxycarbonyl
show the reaction diagram
UDP-N-acetyl-D-galactosamine + glucuronylgalactosyl glycosides
UDP + N-acetyl-D-galactosaminyl-1,4-beta-D-glucuronylgalactosyl glycosides
show the reaction diagram
UDP-N-acetyl-D-galactosamine + glucuronylgalactosyl glycosides
UDP + N-acetyl-D-galactosaminylglucuronylgalactosyl glycosides
show the reaction diagram
additional information
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GalNAcT activity of the recombinant enzyme is determined using a trisaccharide analogue of the linkage region linked to a chromophoric aglycone, GlcA-beta1,3-Gal-beta1,3-Gal-O-methoxyphenyl, i.e. GlcA-Gal-Gal-OMP, as a acceptor substrate, Km is 3.8 mM, and with only the trisaccharide GlcA-beta1,3-Gal-beta1,3-Gal, Km 3.39 mM, no activity with Gal-Gal-OMP. The enzyme activity is even higher with 4- and 6-sulfated GlcA-Gal-Gal-OMP, kM values are 0.6 mM and 1.0 mm, respectively, no activity with 6-disulfated GlcA-Gal-Gal-OMP
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NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
UDP-N-acetyl-D-galactosamine + beta-D-glucuronyl-(1->3)-D-galactosyl-proteoglycan
UDP + N-acetyl-D-galactosaminyl-(1->4)-beta-D-glucuronyl-(1->3)-beta-D-galactosylproteoglycan
show the reaction diagram
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?
UDP-N-acetyl-D-galactosamine + glucuronylgalactosyl glycosides
UDP + N-acetyl-D-galactosaminylglucuronylgalactosyl glycosides
show the reaction diagram
METALS and IONS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
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Manually annotated by BRENDA team
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articular
Manually annotated by BRENDA team
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low content
Manually annotated by BRENDA team
melanoma cell line
Manually annotated by BRENDA team
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very high content
Manually annotated by BRENDA team
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chondrosarcoma cell line
Manually annotated by BRENDA team
thyroid cancer cell line
Manually annotated by BRENDA team
low content
Manually annotated by BRENDA team
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low content
Manually annotated by BRENDA team
myeloma cell line
Manually annotated by BRENDA team
additional information
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immunohistochemic analysis of enzyme content and localization, overview
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
70000
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x * 70000, about, recombinant His-tagged soluble forms of wild-type ChGn-1 and S126LChGn-1 mutant enzymes, SDS-PAGE
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
glycoprotein
Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
recombinant His-tagged soluble forms of wild-type ChGn-1 and S126LChGn-1 mutant from COS-1 cellsby nickel affinity chromatography
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Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
DNA sequence determination and analysis, expression of a truncated form, consisting of the putative catalytic domain, in Spodoptera frugiperda Sf21 insect cells as a soluble FLAG-tagged protein
DNA sequence determination, genomic organization and chromosome localization, functional expression as soluble protein fused to protein A IgG-binding domain in COS-1 cells, secretion into the medium
gene ChGn1, expression of His-tagged wild-type ChGn-1 and soluble mutant enzymes in COS-1 cells, secretion to the medium
gene CSGALNACT1, genotyping and polymorphisms, recombinant expression of His-tagged soluble forms of wild-type ChGn-1 and S126LChGn-1 mutant in COS-1 cells, the enzymes are secreted
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gene CSGALNACT1, quantitative real-time PCR enzyme expression analysis
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LTC cells from rat chondrosarcoma are transfected with chondroitin sulfate N-acetylgalactosaminyltransferase-1 expression plasmid (about 80fold increase of chondroitin sulfate N-acetylgalactosaminyltransferase-1 RNA), metabolic albeling with [35S]sulfate show up to a 2.2fold increase in chondroitin sulfate levels in these cells, but the mRNA level of aggrecan core protein does not change. Aggrecan obained from chondroitin sulfate N-acetylgalactosaminyltransferase-1 overexpressing cells contain a larger amount of chondroitin sulfate, but the chondroitin sulfate chain length is similar to mock-transfected cells.
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Myc-tagged CSGalNAcT-1 expression in HeLa cells
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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
the enzyme is downregulated in Kashin-Beck disease (KBD), an endemic degenerative osteoarthritis, and in primary osteoarthritis, both associated with extracellular matrix degradation
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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
H234R
naturally occuring mutation in exon 5 of gene ChGn1, the mutant shows neuropathy, Bellís palsy and/or hereditary motor and sensory neuropathy, the mutant enzyme is inactive
M509R
naturally occuring mutation in exon 10 of gene ChGn1, the mutant shows neuropathy, Bellís palsy and/or hereditary motor and sensory neuropathy, the mutant enzyme is inactive
S126L
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naturally occuring inactive ChGN1 mutant