GGDB:

EXT2	Last Update:2021-12-17

Keyword: Hereditary multiple exostoses , heparan sulfate , heparin , proteoglycan

Glucuronyltransferase N-Acetylglucosaminyltransferase

GGDB Symbol

EXT2 Alias

Designation

heparan sulfate GlcA/GlcNAc transferase 2, glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase, N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase 2

Organism

Homo sapiens

GeneID

2132

HGNC

EXT2 (3513)

mRNA

NM_000401

map

11p12-p11

Protein

NP_000392

EC#

2.4.1.225

CAZy

GT64

GlyCosmos Glycogenes

EXT2

OMIM

608210

Disease name

GDGDB

Human Protein Atlas

ENSG00000151348

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Orthologous Gene

Mus musculus

mRNA	NM_010163
Protein	NP_034293
MGI	29453

Rattus norvegicus

mRNA	XM_230303
Protein	XP_230303

Drosophila melanogaster

mRNA	NM_176188
Protein	NP_788368

Acceptor Substrates (Reference)

Proc. Natl. Acad. Sci. U.S.A. 2000-01-01 ;97(2):668-73.

The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate.

McCormick C. et al. PMID:10639137

Proc. Natl. Acad. Sci. U.S.A. 2003-10-01 ;278(43):41618-23.

In vitro heparan sulfate polymerization: crucial roles of core protein moieties of primer substrates in addition to the EXT1-EXT2 interaction.

Kim BT. et al. PMID:12907685 Refer to: TABLE I

DONOR : UDP-GalNAc UDP-GlcUA

Glc	Man	Gal	GlcNAc	GalNAc	Fuc	Xyl	Neu5Ac	Neu5Gc	GlcA	IdoA	GlcN

Substrate Transferred

	JCGG-STR003511 GlyTouCan: G99041PQ GlyCosmos Glycans: G99041PQ	UDP-GalNAc	EXT1_6

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV	UDP-GlcUA	EXT1_5

Substrate Not Transferred

	EXT1_1

	EXT1_2

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	JCGG-STR003511 GlyTouCan: G99041PQ GlyCosmos Glycans: G99041PQ

	EXT1_1

	EXT1_2

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	EXT1_1

	EXT1_2

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	JCGG-STR003511 GlyTouCan: G99041PQ GlyCosmos Glycans: G99041PQ

table 1
Glycoprotein	Enzyme activity
a-Thrombomodulin coexpression of EXT1 and EXT2	54200
Glypican coexpression of EXT1 and EXT2	28600
Glypican-1	0
a-Thrombomodulin	0
Glypican-1 mixture of EXT1 and EXT2	0
a-Thrombomodulin mixture of EXT1 and EXT2	0

J. Biol. Chem. 1998-10-01 ;273(41):26265-8.

The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate.

Lind T. et al. PMID:9756849

Acceptor Substrates (KEM-C)

Expression

RNA Expression from Human Protein Atlas

This RNA expression data uses "HPA data" from the Human Protein Atlas. Tissue RNA Expression

data available from v22.proteinatlas.org

Expression (Reference)

Proc. Natl. Acad. Sci. U.S.A. 1996-10-01 ;5(10):1547-57.

Positional cloning of a gene involved in hereditary multiple exostoses.

Wuyts W. et al. PMID:8894688

brainheartkidneyliverlungpancreasplacentaskeletal muscle

Gene Ontology

Provided by GOA

Function
N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity
acetylglucosaminyltransferase activity
glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity
glucuronosyltransferase activity
heparan sulfate N-acetylglucosaminyltransferase activity
metal ion binding
protein binding
protein heterodimerization activity
protein homodimerization activity
transferase activity, transferring glycosyl groups

Process
cell differentiation
cellular polysaccharide biosynthetic process
glycosaminoglycan biosynthetic process
heparan sulfate proteoglycan biosynthetic process
heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process
mesoderm formation
ossification
protein glycosylation
signal transduction

Component
Golgi apparatus
Golgi membrane
UDP-N-acetylglucosamine transferase complex
endoplasmic reticulum
endoplasmic reticulum membrane
extracellular exosome
integral component of membrane
membrane

This page does not indicate all of the enzymatic reaction, and expression of "EXT2".