GGDB:

EXTL3 encodes exostosin-like glycosyltransferase 3. This gene belongs to the multiple exostoses (EXT) family and is involved in heparan sulfate and heparin biosynthesis. EXTL3 has the activity to transfer GlcNAc to both the linkage tetrasaccharide GlcA in the initiation of heparan sulfate synthesis and the non-reducing terminal GlcA in the elongation process via α1,4 binding activity. Phenotypes of mice lacking the EXTL3 gene was embryonic lethal at E10.5.

Keyword: heparan sulfate , heparin , proteoglycan

N-Acetylglucosaminyltransferase

GGDB Symbol

EXTL3 Alias

Designation

heparan sulfate GlcA/GlcNAc transferase like 3

Organism

Homo sapiens

GeneID

2137

HGNC

EXTL3 (3518)

mRNA

NM_001440

map

8p21

Protein

NP_001431

EC#

2.4.1.224

CAZy

GT64

GlyCosmos Glycogenes

EXTL3

OMIM

605744

Disease name

GDGDB

Human Protein Atlas

ENSG00000012232

Mus musculus

mRNA	NM_018788
Protein	NP_061258
MGI	47105

Rattus norvegicus

mRNA	NM_020097
Protein	NP_064482

Caenorhabditis elegans

mRNA	NM_066967
Protein	NP_499368

Drosophila melanogaster

mRNA	NM_079066
Protein	NP_523790

Biochem. Biophys. Res. Commun. 2001-06-01 ;98(13):7176-81.

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.

Kim BT. et al. PMID:11390981 Refer to: Table 1.

DONOR : UDP-GlcNAc

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

Substrate Transferred

	JCGG-STR003511 GlyTouCan: G99041PQ GlyCosmos Glycans: G99041PQ	UDP-GlcNAc	EXTL3_1

Substrate Not Transferred

	JCGG-STR027487 GlyTouCan: G14371GV GlyCosmos Glycans: G14371GV

	JCGG-STR032618 GlyTouCan: G43702JT GlyCosmos Glycans: G43702JT

	EXT1_3

	JCGG-STR032618 GlyTouCan: G43702JT GlyCosmos Glycans: G43702JT

	JCGG-STR028206 GlyTouCan: G87877ES GlyCosmos Glycans: G87877ES

	EXT1_4

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

Proc. Natl. Acad. Sci. U.S.A. 1998-02-01 ;243(1):61-6.

Structure, chromosomal location, and expression profile of EXTR1 and EXTR2, new members of the multiple exostoses gene family.

Saito T. et al. PMID:9473480

Genomics 1998-01-01 ;47(2):230-7.

Identification of a third EXT-like gene (EXTL3) belonging to the EXT gene family.

Van Hul W. et al. PMID:9479495

braincolonheartkidneyliverlungovarypancreasperipheral blood leukocyteplacentaprostateskeletal musclesmall intestinespleentestisthymus

Provided by GOA

Function
glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity
metal ion binding
receptor activity

Process
IRE1-mediated unfolded protein response
glycosaminoglycan biosynthetic process
heparan sulfate proteoglycan biosynthetic process
positive regulation of cell growth

Component
endoplasmic reticulum
endoplasmic reticulum membrane
integral component of membrane

EXTL3

N-Acetylglucosaminyltransferase

Orthologous Gene

Mus musculus

Rattus norvegicus

Caenorhabditis elegans

Drosophila melanogaster

Acceptor Substrates (Reference)

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.

DONOR : UDP-GlcNAc

Substrate Transferred

Substrate Not Transferred

Acceptor Substrates (KEM-C)

Expression

Expression (Reference)

Structure, chromosomal location, and expression profile of EXTR1 and EXTR2, new members of the multiple exostoses gene family.

Identification of a third EXT-like gene (EXTL3) belonging to the EXT gene family.

Gene Ontology