2.4.1.226: N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase
This is an abbreviated version!
For detailed information about N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase, go to the full flat file.
Word Map on EC 2.4.1.226
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2.4.1.226
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glycosaminoglycans
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proteoglycans
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tetrasaccharide
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dermatan
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polymerizing
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csgalnact1
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hyperelongation
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brachydactyly
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glcua
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unsulfated
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preaxial
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medicine
- 2.4.1.226
- glycosaminoglycans
- proteoglycans
- tetrasaccharide
- dermatan
-
polymerizing
- csgalnact1
-
hyperelongation
- brachydactyly
-
glcua
-
unsulfated
-
preaxial
- medicine
Reaction
Synonyms
AcbD, ApCS, bifunctional CS synthase, ChGn-1, ChGn-2, chondroitin beta-glucuronyltransferase, chondroitin glucuronyltransaferase II, chondroitin glucuronyltransferase, chondroitin glucuronyltransferase II, chondroitin sulfate beta-1,4-N-acetylgalactosaminyltransferase-1, chondroitin sulfate glucoronyltransferase, chondroitin sulfate glucuronyltransferase, chondroitin sulfate glucuronyltransferase/chondroitin synthase-3, chondroitin sulfate N-acetylgalactosaminyltransferase-1, chondroitin sulfate N-acetylgalactosaminyltransferase-2, chondroitin sulfate synthase 1, chondroitin sulfate synthase-1, chondroitin sulfate synthase-2, chondroitin sulfate synthase-3, chondroitin synthase, chondroitin synthase-3, CHPF, ChSy, ChSy-1, ChSy-3, CHSY1, CHSY2, CHSY3, CS N-acetylgalactosaminyltransferase-1, CS synthase 1, CSGalNAcT-1, CSGlcA-T, CSGlcAT, CSGlcAT-II, CSGlcAT/ChSy-3, CSS1, CSS2, GlcAT-II, glucuronyltransferase, uridine diphosphoglucuronate-chondroitin, glucuronyltransferase-II, More, UDP-glucuronate:chondroitin glucuronyltransferase
ECTree
2.4.1.226 N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferaseAdvanced search results
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results (Engineering
Engineering on EC 2.4.1.226 - N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase
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PROTEIN VARIANTS 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
D184A
mutant protein which lacks any glycosyltransferase activity but interacts with other ChSy family members shows that the glycosyltransferase activity of CSGlcA-T plays an important role in chondroitin polymerization
F362S
naturally occuring missense mutation of the enzyme activity-related, highly conserved residue, the mutant protein has a 56% decrease in GalNAcT-II activity and a 55% decrease in GlcAT-II activity compared to the wild-type
S126L
naturally occuring mutation, inactive mutant, the amount of chondroitin sulfate proteoglycans is reduced. In men, multiple sclerosis patients with S126L have a slower disease progression. This cSNP might be associated with the gender differences in clinical course of multiple sclerosis
additional information
additional information
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adenoviral gene delivery of the enzyme into intervertebral discs of 4-month -old ICR mice displays a substantial increase in the level of chondroitin sulfate biosynthesis
additional information
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overexpression of CSGlcA-T increases the amount of chondroitin sulfate in HeLa cells, whereas the RNA interference of CSGlcA-T results in a reduction of the amount of chondroitin sulphate in the cells
additional information
overexpression of CSGlcA-T increases the amount of chondroitin sulfate in HeLa cells, whereas the RNA interference of CSGlcA-T results in a reduction of the amount of chondroitin sulphate in the cells
additional information
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overexpression of CSS3 increases the amount of chondroitin sulfate in HeLa cells, while the RNA interference of CSS3 results in a reduction in the amount of CS in the cells
additional information
overexpression of CSS3 increases the amount of chondroitin sulfate in HeLa cells, while the RNA interference of CSS3 results in a reduction in the amount of CS in the cells
additional information
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overexpression of the enzyme in chondrocytic cells further enhances chondroitin sulfate biosynthesis but not that of the aggrecan core protein, indicating that the enzyme activity is not saturated in the cells and that aggrecan synthesized in the overexpressing cells is heavier than the native molecule
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two missense mutations in the CHSY1 gene in patients with neuropathy. These mutations are associated with a profound decrease in enzyme activity. Construction of a soluble form of ChSy-1
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both CHSY1 expression and CS56 staining are dramatically decreased in CHSY1-silenced cells
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both CHSY1 expression and CS56 staining are dramatically decreased in CHSY1-silenced cells
additional information
deletion of the ChGn-2 gene significantly reduces LDL retention in the DIT mouse model. Chondroitin sulfate N-acetylgalactosaminyltransferase-2 deletion alleviates lipoprotein retention in early atherosclerosis and attenuates aortic smooth muscle cell migration. A functional assay of ASMCs prepared from ChGn-2-/- mice displays abrogation of platelet-derived growth factor (PDGF)-mediated migration via reduced PDGF receptor phosphorylation. Evaluation of LDL retention in a diffuse intimal thickening (DIT) model using partial carotid ligation on ChGn-2/LDL receptor double knockout (ChGn-2-/-/LDLr-/-) mice
additional information
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deletion of the ChGn-2 gene significantly reduces LDL retention in the DIT mouse model. Chondroitin sulfate N-acetylgalactosaminyltransferase-2 deletion alleviates lipoprotein retention in early atherosclerosis and attenuates aortic smooth muscle cell migration. A functional assay of ASMCs prepared from ChGn-2-/- mice displays abrogation of platelet-derived growth factor (PDGF)-mediated migration via reduced PDGF receptor phosphorylation. Evaluation of LDL retention in a diffuse intimal thickening (DIT) model using partial carotid ligation on ChGn-2/LDL receptor double knockout (ChGn-2-/-/LDLr-/-) mice
additional information
generation of chondroitin sulfate (CS) N-acetylgalactosaminyltransferase-1 (T1) gene knockout (KO) mice, Ehlers-Danlos syndrome (EDS)-like phenotype and phenotypic features, histological analysis, detailed overview. The protein levels of collagen type 1 and Wnt3a are significantly decreased by T1 gene knockdown by T1KD. Downregulation of collagen type 1 and Wnt3a by T1 gene knockdown in vitro and in vivo
additional information
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deletion of the ChGn-2 gene significantly reduces LDL retention in the DIT mouse model. Chondroitin sulfate N-acetylgalactosaminyltransferase-2 deletion alleviates lipoprotein retention in early atherosclerosis and attenuates aortic smooth muscle cell migration. A functional assay of ASMCs prepared from ChGn-2-/- mice displays abrogation of platelet-derived growth factor (PDGF)-mediated migration via reduced PDGF receptor phosphorylation. Evaluation of LDL retention in a diffuse intimal thickening (DIT) model using partial carotid ligation on ChGn-2/LDL receptor double knockout (ChGn-2-/-/LDLr-/-) mice
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additional information
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gene silencing via shRNA sequences targeting Rattus norvegicus Smad2, Smad3, CHSY1, c-jun and Sp1, the shRNAs are transfected using a lentiviral vector
