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Information on EC 2.4.1.223 - glucuronosyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
for references in articles please use BRENDA:EC2.4.1.223
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EC Tree 2 Transferases
2.4 Glycosyltransferases
2.4.1 Hexosyltransferases
2.4.1.223 glucuronosyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
IUBMB Comments
Enzyme involved in the initiation of heparin and heparan sulfate synthesis, transferring GlcNAc to the (GlcA-Gal-Gal-Xyl-)Ser core. Apparently products of both the human EXTL2 and EXTL3 genes can catalyse this reaction. In Caenorhabditis elegans, the product of the rib-2 gene displays this activity as well as that of EC 2.4.1.224, glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-α-N-acetylglucosaminyltransferase. For explanation of the use of a superscript in the systematic name, see 2-Carb-37.2.
The enzyme appears in viruses and cellular organisms
- 2.4.1.223
- heparan
- proteoglycans
- glycosyltransferases
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ext-like
- glycosaminoglycans
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extls
- chondroitin
- exostosins
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mucous
- n-acetylgalactosamine
- tetrasaccharide
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wingless
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decapentaplegic
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brunner
- heparitinase
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pancreaticobiliary
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hspgs
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fam20b
Reaction Schemes
showSynonyms
extl2, alpha1,4-n-acetylglucosaminyltransferase, brother of tout-velu, exostosin-like 2, galntl-1, alpha-n-acetylglucosaminyltransferase i, more
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
alpha-N-acetylglucosaminyltransferase I
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alpha1,4-N-acetylglucosaminyltransferase
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EXTL2
glucuronosylgalactosylproteoglycan 4-alpha-N-acetylglucosaminyltransferase
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
UDP-N-acetyl-alpha-D-glucosamine + [protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine = UDP + [protein]-3-O-(alpha-D-GlcNAc-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
hexosyl group transferase
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PATHWAY SOURCE
PATHWAYS
MetaCyc
heparan sulfate biosynthesis
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SYSTEMATIC NAME
IUBMB Comments
UDP-N-acetyl-alpha-D-glucosamine:[protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine 4IV-alpha-N-acetyl-D-glucosaminyltransferase (configuration-retaining)
Enzyme involved in the initiation of heparin and heparan sulfate synthesis, transferring GlcNAc to the (GlcA-Gal-Gal-Xyl-)Ser core. Apparently products of both the human EXTL2 and EXTL3 genes can catalyse this reaction. In Caenorhabditis elegans, the product of the rib-2 gene displays this activity as well as that of EC 2.4.1.224, glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase. For explanation of the use of a superscript in the systematic name, see {iupac/2carb/37#372::2-Carb-37.2}.
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CAS REGISTRY NUMBER
COMMENTARY
145539-84-0
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336193-98-7
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
LITERATURE
COMMENTARY
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
UDP-GlcA + GlcNAc-[GlcA-GlcNAc]4
UDP + [GlcA-GlcNAc]5
Substrates: measurement of GlcAT-II activity
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UDP-GlcNAc + [GlcA-GlcNAc]4
UDP + GlcNAc-[GlcAGlcNAc]4
Substrates: measurement of GlcNAcTII activity, through the ability to extend K5 heparosan oligosaccharides. Reaction is sensitive to EDTA treatment
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UDP-N-acetyl-D-galactosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-O-Ser
UDP + alpha-N-acetyl-D-galactosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-O-Ser
UDP-N-acetyl-D-galactosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-naphthalenemethanol
UDP + alpha-N-acetyl-D-galactosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl1-O-naphthalenemethanol
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Substrates: -
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UDP-N-acetyl-D-galactosamine + beta-D-glucuronosyl-(1-3)-beta-D-N-acetylgalactosamine
UDP + alpha-N-acetyl-D-galactosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-N-acetylgalactosamine
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Substrates: -
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UDP-N-acetyl-D-glucosamine + alpha-thrombomodulin
UDP + alpha-N-acetyl-D-glucosaminyl-alpha-thrombomodulin
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Substrates: 55% of the activity with beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-beta-O-C2H4NHCbz
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-beta-O-Ser-Gly-Trp-Pro-Asp-Gly
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-beta-O-Ser-Gly-Trp-Pro-Asp-Gly
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Substrates: -
Products: 18% of the activity with beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-beta-O-C2H4NHCbz
Products: 18% of the activity with beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-beta-O-C2H4NHCbz
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-beta-O-C2H4NHCbz
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-beta-O-C2H4NHCbz
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Substrates: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-benzyloxycarbonyl
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-benzyloxycarbonyl
UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-naphthalenemethanol
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl1-O-naphthalenemethanol
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Substrates: 28% of the rate with UDP-N-acetyl-D-galactosamine
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UDP-N-acetyl-D-glucosamine + glypican-1
UDP + alpha-N-acetyl-D-glucosaminyl-glypican-1
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Substrates: 30% of the activity with beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-beta-O-C2H4NHCbz
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UDP-N-acetyl-D-galactosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-O-Ser
UDP + alpha-N-acetyl-D-galactosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-O-Ser
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Substrates: 46% of the rate with beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-naphthalenemethanol
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UDP-N-acetyl-D-galactosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-O-Ser
UDP + alpha-N-acetyl-D-galactosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-O-Ser
Substrates: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
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Substrates: initiation and elongation of heparan sulfate
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
Substrates: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
Substrates: essential in the heparan sulfate biosynthesis
Products: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
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Substrates: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
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Substrates: essential in the heparan sulfate biosynthesis
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-benzyloxycarbonyl
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-benzyloxycarbonyl
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Substrates: -
Products: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-benzyloxycarbonyl
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-benzyloxycarbonyl
Substrates: -
Products: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-benzyloxycarbonyl
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-1-O-benzyloxycarbonyl
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Substrates: -
Products: -
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additional information
?
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Substrates: activity is only detectable when enzymes are coexpressed, a mixture of individually expressed enzymes is inactive
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additional information
?
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Substrates: no transfer of N-acetylglucosamine to beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-1-O-Ser
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additional information
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Substrates: N-acetylgalactosaminyltransferase-like 1 specifically interferes with the activity of TGF-beta type II receptor ActR-IIB in vivo
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
LITERATURE
COMMENTARY
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
additional information
?
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Substrates: N-acetylgalactosaminyltransferase-like 1 specifically interferes with the activity of TGF-beta type II receptor ActR-IIB in vivo
Products: -
Products: -
?
UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
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Substrates: initiation and elongation of heparan sulfate
Products: -
Products: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
Substrates: essential in the heparan sulfate biosynthesis
Products: -
Products: -
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UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
UDP + alpha-N-acetyl-D-glucosaminyl-(1-4)-beta-D-glucuronosyl-(1-3)-beta-D-galactosyl-(1-3)-beta-D-galactosyl-(1-4)-beta-D-xylosyl-proteoglycan
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Substrates: essential in the heparan sulfate biosynthesis
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Adenocarcinoma
A novel endo-beta-galactosidase from Clostridium perfringens that liberates the disaccharide GlcNAcalpha 1-->Gal from glycans specifically expressed in the gastric gland mucous cell-type mucin.
Carcinogenesis
Aberrant glycosaminoglycan biosynthesis by tumor suppressor EXTL2 deficiency promotes liver inflammation and tumorigenesis through Toll-like 4 receptor signaling.
Demyelinating Diseases
The glycosyltransferase EXTL2 promotes proteoglycan deposition and injurious neuroinflammation following demyelination.
Exostoses, Multiple Hereditary
Expression of rib-1, a Caenorhabditis elegans homolog of the human tumor suppressor EXT genes, is indispensable for heparan sulfate synthesis and embryonic morphogenesis.
Exostoses, Multiple Hereditary
rib-2, a Caenorhabditis elegans homolog of the human tumor suppressor EXT genes encodes a novel alpha1,4-N-acetylglucosaminyltransferase involved in the biosynthetic initiation and elongation of heparan sulfate.
glucuronosyl-galactosyl-proteoglycan 4-alpha-n-acetylglucosaminyltransferase deficiency
Aberrant glycosaminoglycan biosynthesis by tumor suppressor EXTL2 deficiency promotes liver inflammation and tumorigenesis through Toll-like 4 receptor signaling.
Kashin-Beck Disease
Altered Expression of Aggrecan, FAM20B, B3GALT6, and EXTL2 in Patients with Osteoarthritis and Kashin-Beck Disease.
Mucopolysaccharidoses
Gene silencing of EXTL2 and EXTL3 as a substrate deprivation therapy for heparan sulphate storing mucopolysaccharidoses.
Mucopolysaccharidosis III
Gene silencing of EXTL2 and EXTL3 as a substrate deprivation therapy for heparan sulphate storing mucopolysaccharidoses.
Neoplasms
Aberrant glycosaminoglycan biosynthesis by tumor suppressor EXTL2 deficiency promotes liver inflammation and tumorigenesis through Toll-like 4 receptor signaling.
Neoplasms
Expression of rib-1, a Caenorhabditis elegans homolog of the human tumor suppressor EXT genes, is indispensable for heparan sulfate synthesis and embryonic morphogenesis.
Neoplasms
EXTL2, a Member of the EXT Family of Tumor Suppressors, Controls Glycosaminoglycan Biosynthesis in a Xylose Kinase-dependent Manner.
Neoplasms
rib-2, a Caenorhabditis elegans homolog of the human tumor suppressor EXT genes encodes a novel alpha1,4-N-acetylglucosaminyltransferase involved in the biosynthetic initiation and elongation of heparan sulfate.
Neoplasms
Roles of EXTL2, a member of the EXT family of tumour suppressors, in liver injury and regeneration processes.
Neoplasms
The tumor suppressor EXT-like gene EXTL2 encodes an alpha1, 4-N-acetylhexosaminyltransferase that transfers N-acetylgalactosamine and N-acetylglucosamine to the common glycosaminoglycan-protein linkage region. The key enzyme for the chain initiation of heparan sulfate.
Neuroinflammatory Diseases
The glycosyltransferase EXTL2 promotes proteoglycan deposition and injurious neuroinflammation following demyelination.
Osteoarthritis
Altered Expression of Aggrecan, FAM20B, B3GALT6, and EXTL2 in Patients with Osteoarthritis and Kashin-Beck Disease.
Pancreatic Neoplasms
Clinical utility of quantitative RT-PCR targeted to alpha1,4-N-acetylglucosaminyltransferase mRNA for detection of pancreatic cancer.
Stomach Neoplasms
Usefulness of the real-time reverse transcription-polymerase chain reaction assay targeted to alpha1,4-N-acetylglucosaminyltransferase for the detection of gastric cancer.
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
recombinant enzyme lacking first N-terminal 57 amino acids to give a soluble protein, expression in malignant fibrous histocytoma cell line
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-
brenda
recombinant enzymes EXT1 and EXT2, activity is only detectable when enzymes are coexpressed, a mixture of individually expressed enzymes is inactive
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brenda
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
epithelial breast carcinoma cell line
brenda
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
human EXTL3 is inserted into the Golgi apparatus membrane by an N-terminal transmembrane helix of 20 residues, followed by an unstructured protein segment of about 18 residues, rich in charged amino acids, and then around 80 residues of coiled-coil structure that may play a role in positioning and/or orienting the following catalytic domains on the Golgi membrane
brenda
Highest Expressing Human Cell Lines
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
physiological function
physiological function
the EXTL3 enzyme mainly works as an initiator in heparan sulfate biosynthesis
physiological function
EXTL2 functions in chain termination of heparan sulfate. EXTL2 controls FGF2 signaling through regulation of heparan sulfate biosynthesis
physiological function
EXTL2-/- mice have exacerbated axonal damage and myelin disruption relative to wild-type mice, and increased representation of microglia/macrophages within lesions. Increased accumulation of microglia and macrophages is found within lesions of EXTL2-/- animals. Activated bone marrow-derived macrophages from EXTL2-/- mice overproduce tumor necrosis factor alpha and matrix metalloproteinases. A demyelinating injury to the spinal cord of EXTL2/- mice results in excessive deposition of chondroitin sulfate proteoglycans in the lesion area
physiological function
EXTL3 initiates the synthesis of heparan sulfate (HS) chains in detriment of chondroitin sulfate (CS) biosynthesis, regulating the balance of the HS/CS ratio in cells. Deletion of EXTL3 leads to the complete depletion of HS, both at the cell surface and total cell content, and an aberrant glycosylation of SDC4
physiological function
EXTL2 functions as a negative regulator of HS biosynthesis, with impact over the glycoproteome of gastric cancer cells. Knockout of EXTL2 enhances HS levels along with concomitant upregulation of Syndecan-4. EXTL2 knockout leads to a more motile and invasive phenotype in MKN74 gastric cells
Show AA Sequence and Transmembrane Helices (1963 entries)
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PDB
SCOP
CATH
UNIPROT
ORGANISM
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SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
dimer
EXTL3's globular domain forms a symmetrical homodimer, cryo-EM data. The GT47-family glycosyltransferase domains exhibit a GT-B-type fold with a conserved Asp/Glu located on the Calpha4 helix. The EXTL3 GT64 domain active site is separated from the potential active site of the GT47 domain by a substantial distance
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CRYSTALLIZATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
additional information
-
enzyme null mutant, defects in Hedgehog, Wingless and Decapentaplegic signalling
additional information
-
activity is only detectable when enzymes EXT1 and EXT2are coexpressed, a mixture of individually expressed enzymes is inactive
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TEMPERATURE STABILITY
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
recombinant enzyme lacking first N-terminal 57 amino acids to give a soluble protein, expression in malignant fibrous histiocytoma cell line
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CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
expression in COS-1 cells, lacking the putative NH2-terminal transmembrane and cytoplasmic domains
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expression in COS-1 cells, truncated form , lacking the first 58 N-terminal amino acids
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human lumenal N-glycosylated EXTL3 (EXTL3DELTAN) is cloned, expressed in human embryonic kidney cells
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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
EXTL2 is upregulated in MDA-MB-231 and HCC38 epithelial breast carcinoma cell lines
the expression of EXTL2 is highly upregulated in MDA-MB-231 cells, and HCC38 cells
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APPLICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
medicine
medicine
EXTL3 mRNA and protein are poorly expressed in prostate cancer samples, with worse overall survival. EXTL3 is markedly linked with signaling pathways in prostate cancer by GSEA, including calcium, chemokine, ERBB, JAK STAT, MAPK, WNT, oxidative phosphorylation pathways. EXTL3 expression is significantly associated with MSI, immune cells, immune checkpoint molecules, tumor microenvironment and immune cells infiltration
medicine
EXTL2 knockout leads to a more motile and invasive phenotype in MKN74 gastric cells
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Kitagawa, H.; Egusa, N.; Tamura, J.I.; Kusche-Gullberg, M.; Lindahl, U.; Sugahara, K.
rib-2, a Caenorhabditis elegans homolog of the human tumor suppressor EXT genes encodes a novel alpha-1,4-N-acetylglucosaminyltransferase involved in the biosynthetic initiation and elongation of heparan sulfate
J. Biol. Chem.
276
4834-4838
2001
Caenorhabditis elegans
brenda
Kim, B.T.; Kitagawa, H.; Tamura, J.I.; Saito, T.; Kusche-Gullberg, M.; Lindahl, U.; Sugahara, K.
Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha-1,4-N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/heparin biosynthesis
Proc. Natl. Acad. Sci. USA
98
7176-7181
2001
Homo sapiens
brenda
Kim, B.T.; Kitagawa, H.; Tamura, J.I.; Kusche-Gullberg, M.; Lindahl, U.; Sugahara, K.
Demonstration of a novel gene DEXT3 of Drosophila melanogaster as the essential N-acetylglucosamine transferase in the heparan sulfate biosynthesis: Chain initiation and elongation
J. Biol. Chem.
277
13659-13665
2002
Drosophila melanogaster (Q9XZ08)
brenda
Han, C.; Belenkaya, T.Y.; Khodoun, M.; Tauchi, M.; Lin, X.; Lin, X.
Distinct and collaborative roles of Drosophila EXT family proteins in morphopgen signalling and gradient formation
Development
131
1563-1575
2005
Drosophila melanogaster
brenda
Kitagawa, H.; Shimakawa, H.; Sugahara, K.
The tumor suppressor EXT-like gene EXTL2 encodes an alpha1,4N-acetylhexosaminyltransferase that transfers N-acetylgalactosamine and N-acetylglucosamine to the common glucosaminoglycan-protein linkage region. The key enzyme for the chain initiation of heparan sulfate
J. Biol. Chem.
274
13933-13937
1999
Homo sapiens
brenda
Kim, B.T.; Kitagawa, H.; Tanaka, J.; Tamura, J.I.; Sugahara, K.
In vitro heparan sulfate polymerization: crucial roles of core protein moieties of primer substrates in addition to the EXT1-EXT2 interaction
J. Biol. Chem.
278
41618-41623
2003
Homo sapiens
brenda
Herr, P.; Korniychuk, G.; Yamamoto, Y.; Grubisic, K.; Oelgeschlaeger, M.
Regulation of TGF-beta signalling by N-acetylgalactosaminyltransferase-like 1
Development
135
1813-1822
2008
Xenopus laevis (Q6PA81)
brenda
Awad, W.; Kjellstroem, S.; Svensson Birkedal, G.; Mani, K.; Logan, D.T.
Structural and biophysical characterization of human EXTL3 domain organization, glycosylation, and solution structure
Biochemistry
57
1166-1177
2018
Homo sapiens (O43909)
brenda
Nadanaka, S.; Kitagawa, H.
Exostosin-like 2 regulates FGF2 signaling by controlling the endocytosis of FGF2
Biochim. Biophys. Acta Gen. Subj.
1862
791-799
2018
Mus musculus (Q9ES89), Mus musculus
brenda
Sembajwe, L.; Katta, K.; Gronning, M.; Kusche-Gullberg, M.
The exostosin family of glycosyltransferases MRNA expression profiles and heparan sulphate structure in human breast carcinoma cell lines
Biosci. Rep.
38
1-12
2018
Homo sapiens (O43909), Homo sapiens (Q9UBQ6), Homo sapiens
brenda
Chang, P.; Chen, S.; Chang, X.; Zhu, J.; Tang, Q.; Ma, L.
EXTL3 could serve as a potential biomarker of prognosis and immunotherapy for prostate cancer and its potential mechanisms
Eur. J. Med. Res.
27
115
2022
Homo sapiens (O43909)
brenda
Marques, C.; Pocas, J.; Gomes, C.; Faria-Ramos, I.; Reis, C.A.; Vives, R.R.; Magalhaes, A.
Glycosyltransferases EXTL2 and EXTL3 cellular balance dictates heparan sulfate biosynthesis and shapes gastric cancer cell motility and invasion
J. Biol. Chem.
298
102546
2022
Homo sapiens (O43909), Homo sapiens (Q9UBQ6)
brenda
Pu, A.; Mishra, M.K.; Dong, Y.; Ghorbanigazar, S.; Stephenson, E.L.; Rawji, K.S.; Silva, C.; Kitagawa, H.; Sawcer, S.; Yong, V.W.
The glycosyltransferase EXTL2 promotes proteoglycan deposition and injurious neuroinflammation following demyelination
J. Neuroinflammation
17
220
2020
Mus musculus (Q9ES89)
brenda
Wilson, L.F.L.; Dendooven, T.; Hardwick, S.W.; Echevarria-Poza, A.; Tryfona, T.; Krogh, K.B.R.M.; Chirgadze, D.Y.; Luisi, B.F.; Logan, D.T.; Mani, K.; Dupree, P.
The structure of EXTL3 helps to explain the different roles of bi-domain exostosins in heparan sulfate synthesis
Nat. Commun.
13
3314
2022
Homo sapiens (O43909)
brenda
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