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Information on EC 2.4.1.224 - glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase and Organism(s) Drosophila melanogaster

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IUBMB Comments
Involved in the biosynthesis of heparin and heparan sulfate. Some forms of the enzyme from human (particularly the enzyme complex encoded by the EXT1 and EXT2 genes) act as bifunctional glycosyltransferases, which also have the 4-beta-glucuronosyltransferase (EC 2.4.1.225, N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase) activity required for the synthesis of the heparan sulfate disaccharide repeats. Other human forms of this enzyme (e.g. the product of the EXTL1 gene) have only the 4-alpha-N-acetylglucosaminyltransferase activity. In Caenorhabditis elegans, the product of the rib-2 gene displays the activities of this enzyme as well as EC 2.4.1.223, glucuronosyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase.
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Drosophila melanogaster
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The taxonomic range for the selected organisms is: Drosophila melanogaster
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Synonyms
exostosin-1, exostosin-2, tout-velu, sister of tout-velu, heparan sulfate polymerase, exostosin1, sister of tout velu, exostosin2, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
alpha-N-acetylglucosaminyltransferase II
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glucuronyl-N-acetylglucosaminoproteoglycan 4-alpha-N-acetylglucosaminyltransferase
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heparan sulfate polymerase
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N-acetyl-D-glucosaminyl-(N-acetyl-D-glucosamine) transferase
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sister of tout velu
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sister of tout-velu
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c.f. EC 2.4.1.225
tout-velu
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c.f. EC 2.4.1.225
ttv
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c.f. EC 2.4.1.225
UDP-GlcNAc:oligosaccharide beta-N-acetylglucosaminyltransferase
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-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
transfer of alpha-N-acetylglucosamine
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SYSTEMATIC NAME
IUBMB Comments
UDP-N-acetyl-D-glucosamine:beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
Involved in the biosynthesis of heparin and heparan sulfate. Some forms of the enzyme from human (particularly the enzyme complex encoded by the EXT1 and EXT2 genes) act as bifunctional glycosyltransferases, which also have the 4-beta-glucuronosyltransferase (EC 2.4.1.225, N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase) activity required for the synthesis of the heparan sulfate disaccharide repeats. Other human forms of this enzyme (e.g. the product of the EXTL1 gene) have only the 4-alpha-N-acetylglucosaminyltransferase activity. In Caenorhabditis elegans, the product of the rib-2 gene displays the activities of this enzyme as well as EC 2.4.1.223, glucuronosyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase.
CAS REGISTRY NUMBER
COMMENTARY hide
145539-84-0
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336193-98-7
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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-glucosamine + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan
UDP + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan
show the reaction diagram
UDP-N-acetyl-D-glucosamine + N-acetylheparosan oligosaccharide
?
show the reaction diagram
with terminal nonreducing glucuronic acid
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-
?
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-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan
UDP + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan
show the reaction diagram
elongation of growing chains of heparin and heparan sulfate, tumor suppressor
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-
?
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
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identical localization of sotv and ttv
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
mutations in the tumor suppressor genes EXT1 and EXT2 disturb heparan sulfate proteoglycan biosynthesis and cause multiple osteochondroma. A reduction in Rti shifts the steady-state distribution of EXTs to the trans-Golgi. These accumulated EXTs tend to be degraded and their re-entrance towards the route for polymerizing GAG chains is disengaged. Conversely, EXTs are mislocalized towards the transitional endoplasmic reticulum/cis-Golgi when Rti is overexpressed. Both loss of function and overexpression of rti result in incomplete heparan sulfate proteoglycans and perturb Hedgehog signaling
physiological function
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
EXT1_DROME
760
1
87309
Swiss-Prot
Mitochondrion (Reliability: 5)
EXT2_DROME
717
1
82726
Swiss-Prot
Secretory Pathway (Reliability: 5)
A0A4P7VB75_DROME
772
1
88779
TrEMBL
Mitochondrion (Reliability: 5)
D5SHU8_DROME
299
0
34443
TrEMBL
other Location (Reliability: 3)
A0A0B4KER9_DROME
760
1
87309
TrEMBL
Mitochondrion (Reliability: 5)
EXT3_DROME
972
1
111000
Swiss-Prot
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SUBUNIT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
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formation of heterooligomers of ttv and sotv, co-immunoprecipitation experiments
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
GENERAL STABILITY
ORGANISM
UNIPROT
LITERATURE
Rti regulates the stability of EXT isozymes
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
expression of soluble form in COS-1 cells
gene EXT1, recombinant expression of Myc-DKK-tagged enzyme in S2 cells, interaction analysis with GOLPH3 via yeast two-hybrid assay
gene EXT2, recombinant expression of Myc-DKK-tagged enzyme in S2 cells, interaction analysis with GOLPH3 via yeast two-hybrid assay
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
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)
Manually annotated by BRENDA team
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
Manually annotated by BRENDA team
Bornemann, D.J.; Duncan, J.E.; Staatz, W.; Selleck, S.; Warrior, R.
Abrogation of heparan sulfate synthesis in Drosophila disrupts the Wingless, Hedgehog and Decapentaplegic signaling pathways
Development
131
1927-198
2004
Drosophila melanogaster
Manually annotated by BRENDA team
Chang, W.L.; Chang, C.W.; Chang, Y.Y.; Sung, H.H.; Lin, M.D.; Chang, S.C.; Chen, C.H.; Huang, C.W.; Tung, K.S.; Chou, T.B.
The Drosophila GOLPH3 homolog regulates the biosynthesis of heparan sulfate proteoglycans by modulating the retrograde trafficking of exostosins
Development
140
2798-2807
2013
Drosophila melanogaster (Q9V730), Drosophila melanogaster (Q9Y169)
Manually annotated by BRENDA team