2.4.1.224: glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase

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For detailed information about glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase, go to the full flat file.

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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 Transferases

         2.4 Glycosyltransferases

             2.4.1 Hexosyltransferases

                2.4.1.224 glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase

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1	Activating Compound
319	AA Sequence
1	Application
2	CAS Registry Number
16	Cloned(Commentary)
76	Disease/ Diagnostics
3	Expression
32	General Information
1	General Stability
8	KM Value [mM]
23	Localization
2	Metals/Ions
15	Molecular Weight [Da]
24	Natural Substrates/ Products (Substrates)
253	Organism
3	Pathway
10	PDB ID
1	pH Optimum
2	pH Range
3	pI Value
8	Posttranslational Modification
23	Protein Variants
5	Purification (Commentary)
1	Reaction
1	Reaction Type
51	Reference
38	Source Tissue
1	Specific Activity [micromol/min/mg]
78	Substrates and Products (Substrate)
6	Subunits
58	Synonyms
1	Systematic Name

Systematic Name

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Systematic Name on EC 2.4.1.224 - glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase

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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.