2.4.1.224: glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
This is an abbreviated version!
For detailed information about glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase, go to the full flat file.
Word Map on EC 2.4.1.224
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2.4.1.224
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proteoglycans
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exostoses
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osteochondroma
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glycosaminoglycans
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glycosyltransferase
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hedgehog
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hspgs
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chondrosarcoma
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cartilaginous
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syndecan-1
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glypicans
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cartilage-capped
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wingless
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perlecan
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metaphyses
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decapentaplegic
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co-polymerase
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medicine
- 2.4.1.224
- proteoglycans
- exostoses
- osteochondroma
- glycosaminoglycans
- glycosyltransferase
-
hedgehog
-
hspgs
-
chondrosarcoma
-
cartilaginous
- syndecan-1
-
glypicans
-
cartilage-capped
-
wingless
- perlecan
-
metaphyses
-
decapentaplegic
-
co-polymerase
- medicine
Reaction
Synonyms
alpha-N-acetylglucosaminyltransferase II, exostosin-1, exostosin-2, Exostosin1, Exostosin2, EXT1, EXT2, extosin, glucuronyl-N-acetylglucosaminoproteoglycan 4-alpha-N-acetylglucosaminyltransferase, heparan sulfate polymerase, heparan sulfate synthesis enzyme, KfiA, More, N-acetyl-D-glucosaminyl-(N-acetyl-D-glucosamine) transferase, sister of tout velu, sister of tout-velu, sotv, tout-velu, ttv, UDP-GlcNAc:oligosaccharide beta-N-acetylglucosaminyltransferase
ECTree
2.4.1.224 glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferaseAdvanced search results
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Reference on EC 2.4.1.224 - glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Sharkey, D.J.; Kornfeld, R.
Identification of an N-acetylglucosaminyltransferase in Dictyostelium discoideum that transfers an intersecting N-acetylglucosamine residue to high mannose oligosaccharides
J. Biol. Chem.
264
10411-10419
1989
Dictyostelium discoideum
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, Homo sapiens
Senay, C.; Lind, T.; Muguruma, K.; Tone, Y.; Kitagawa, H.; Sugahara, K.; Lidholt, K.; Lindahl, U.; Kusche-Gullberg, M.
The EXT1/EXT2 tumor suppressors: catalytic activities and role in heparan sulfate biosynthesis
EMBO Rep.
1
282-286
2000
Bos taurus, Mus musculus, no activity in yeast
Lind, T.; Tufaro, F.; McCormick, C.; Lindahl, U.; Lidholt, K.
The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate
J. Biol. Chem.
273
26265-26268
1998
Mus musculus, Bos taurus (O77783)
Lidholt, K.; Lindahl, U.
Biosynthesis of heparin. The D-glucuronosyl- and N-acetyl-D-glucosaminyltransferase reactions and their relation to polymer modification
Biochem. J.
287
21-29
1992
Bos taurus, Mus musculus
-
Lind, T.; Lindahl, U.; Lidholt, K.
Biosynthesis of heparin/heparan sulfate. Identification of a 70-kDa protein catalyzing both the D-glucuronosyl- and the N-acetyl-D-glucosaminyltransferase reactions
J. Biol. Chem.
268
20705-20708
1993
Bos taurus
Lidholt, K.; Fjelstad, M.; Jann, K.; Lindahl, U.
Biosynthesis of heparin. XXV. Substrate specificities of glucosyltransferases involved in formation of heparin precursor and E. coli K5 capsular polysaccharides
Carbohydr. Res.
255
87-101
1994
Escherichia coli, Mus musculus, Escherichia coli O18:K5
Lind, T.
Enzymes involved in heparan sulfate chain elongation
Trends Glycosci. Glycotechnol.
11
221-225
1999
Bos taurus, Cricetinae
-
Wei, G.; Bai, X.; Gabb, M.M.G.; Bame, K.J.; Koshy, T.I.; Spear, P.G.; Esko, J.D.
Location of the glucuronosyltransferase domain in the heparan sulfate copolymerase EXT1 by analysis of Chinese hamster ovary cell mutants
J. Biol. Chem.
275
27733-27740
2000
Cricetinae, Homo sapiens, Mus musculus
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
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)
McCormic, C.; Leduc, Y.; Martindale, D.; Mattison, K.; Esford, L.E.; Dyer, A.P.; Tufaro, F.
The putative tumor suppressor EXT 1 alters the expression of cell surface heparan sulfate
Nat. Genet.
19
158-161
1998
Cricetinae, Mus musculus
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
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
Wuyts, W.; van Hul, W.
Molecular basis of multiple exostoses: mutations in the EXT1 and EXT2 genes
Hum. Mutat.
15
220-227
2000
Homo sapiens
Busse, M.; Kusche-Gullberg, M.
In vitro polymerization of heparan sulfate backbone by the EXT proteins
J. Biol. Chem.
278
41333-41337
2003
Homo sapiens
McCormick, C.; Duncan, G.; Goutsos, K.T.; Tufaro, F.
The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heapran sulfate
Proc. Natl. Acad. Sci. USA
97
668-673
2000
Bos taurus, Homo sapiens
Chen, M.; Bridges, A.; Liu, J.
Determination of the substrate specificities of N-acetyl-D-glucosaminyltransferase
Biochemistry
45
12358-12365
2006
Escherichia coli
Hecht, J.T.; Hayes, E.; Haynes, R.; Cole, W.G.; Long, R.J.; Farach-Carson, M.C.; Carson, D.D.
Differentiation-induced loss of heparan sulfate in human exostosis derived chondrocytes
Differentiation
73
212-221
2005
Homo sapiens (Q16394), Homo sapiens (Q93063)
Murakami, K.; Namikawa, K.; Shimizu, T.; Shirasawa, T.; Yoshida, S.; Kiyama, H.
Nerve injury induces the expression of EXT2, a glycosyltransferase required for heparan sulfate synthesis
Neuroscience
141
1961-1969
2006
Mus musculus
Roberts, I.S.; Gleadle, J.M.
Familial nephropathy and multiple exostoses with exostosin-1 (EXT1) gene mutation
J. Am. Soc. Nephrol.
19
450-453
2008
Homo sapiens
Osterholm, C.; Barczyk, M.M.; Busse, M.; Gronning, M.; Reed, R.K.; Kusche-Gullberg, M.
Mutation in the heparan sulfate biosynthesis enzyme EXT1 influences growth factor signaling and fibroblast interactions with the extracellular matrix
J. Biol. Chem.
284
34935-34943
2009
Mus musculus
Fischer, S.; Filipek-Gorniok, B.; Ledin, J.
Zebrafish Ext2 is necessary for Fgf and Wnt signaling, but not for Hh signaling
BMC Dev. Biol.
11
53
2011
Danio rerio
Matsumoto, Y.; Matsumoto, K.; Irie, F.; Fukushi, J.; Stallcup, W.B.; Yamaguchi, Y.
Conditional ablation of the heparan sulfate-synthesizing enzyme Ext1 leads to dysregulation of bone morphogenic protein signaling and severe skeletal defects
J. Biol. Chem.
285
19227-19234
2010
Mus musculus, Mus musculus C57BL/6
Wang, Y.; Yang, X.; Yamagata, S.; Yamagata, T.; Sato, T.
Involvement of Ext1 and heparanase in migration of mouse FBJ osteosarcoma cells
Mol. Cell. Biochem.
373
63-72
2013
Mus musculus, Mus musculus BALB/c
Huegel, J.; Mundy, C.; Sgariglia, F.; Nygren, P.; Billings, P.C.; Yamaguchi, Y.; Koyama, E.; Pacifici, M.
Perichondrium phenotype and border function are regulated by Ext1 and heparan sulfate in developing long bones: a mechanism likely deranged in hereditary multiple exostoses
Dev. Biol.
377
100-112
2013
Mus musculus (P97464)
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)
Mooij, H.L.; Cabrales, P.; Bernelot Moens, S.J.; Xu, D.; Udayappan, S.D.; Tsai, A.G.; van der Sande, M.A.; de Groot, E.; Intaglietta, M.; Kastelein, J.J.; Dallinga-Thie, G.M.; Esko, J.D.; Stroes, E.S.; Nieuwdorp, M.
Loss of function in heparan sulfate elongation genes EXT1 and EXT 2 results in improved nitric oxide bioavailability and endothelial function
J. Am. Heart Assoc.
3
e001274
2014
Mus musculus (P70428), Mus musculus (P97464), Homo sapiens (Q16394), Homo sapiens (Q93063)
Farhan, S.M.; Wang, J.; Robinson, J.F.; Prasad, A.N.; Rupar, C.A.; Siu, V.M.; Siu, V.M.; Hegele, R.A.
Old gene, new phenotype: mutations in heparan sulfate synthesis enzyme, EXT2 leads to seizure and developmental disorder, no exostoses
J. Med. Genet.
52
666-675
2015
Homo sapiens (Q93063), Homo sapiens
Zhang, R.; Cao, P.; Yang, Z.; Wang, Z.; Wu, J.L.; Chen, Y.; Pan, Y.
Heparan sulfate biosynthesis enzyme, Ext1, contributes to outflow tract development of mouse heart via modulation of FGF signaling
PLoS ONE
10
e0136518
2015
Mus musculus (P97464)
Wu, Z.; Wang, Y.; Wang, J.; Chen, Y.; Guo, Y.
The role of EXT1 gene mutation and its high expression of calcitonin gene-related peptide in the development of multiple exostosis
Biochem. Biophys. Res. Commun.
505
959-965
2018
Homo sapiens (Q16394)
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 (Q16394), Homo sapiens (Q93063)
Kero, D.; Bilandzija, T.; Arapovic, L.; Vukojevic, K.; Saraga-Babic, M.
Syndecans and enzymes involved in heparan sulfate biosynthesis and degradation are differentially expressed during human dontogenesis
Front. Physiol.
9
732
2018
Homo sapiens (Q16394)
Okolicsanyi, R.K.; Bluhm, J.; Miller, C.; Griffiths, L.R.; Haupt, L.M.
An investigation of genetic polymorphisms in heparan sulfate proteoglycan core proteins and key modification enzymes in an Australian Caucasian multiple sclerosis population
Hum. Genomics
14
18
2020
Homo sapiens (Q16394)
Ushakov, V.; Tsidulko, A.; De La Bourdonnaye, G.; Kazanskaya, G.; Volkov, A.; Kiselev, R.; Kobozev, V.; Kostromskaya, D.; Gaytan, A.; Krivoshapkin, A.; Aidagulova, S.; Grigorieva, E.
Heparan sulfate biosynthetic system is inhibited in human glioma due to EXT1/2 and HS6ST1/2 down-regulation
Int. J. Mol. Sci.
18
2301
2017
Homo sapiens (Q16394), Homo sapiens (Q93063)
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