Reviewed, UniProtKB/Swiss-Prot P70428 (EXT2_MOUSE)

Names and origin

Protein names

Recommended name:
    Exostosin-2
    EC=2.4.1.224
    EC=2.4.1.225
Alternative name(s):
    Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
    Multiple exostoses protein 2 homolog

Gene names

Name:

Ext2

Organism

Mus musculus (Mouse)

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus

Protein attributes

Sequence length	718 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is not processed.
Protein existence	Evidence at protein level.

General annotation (Comments)

Function	Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor.
Catalytic activity	UDP-N-acetyl-D-glucosamine + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan = UDP + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan. UDP-alpha-D-glucuronate + N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan = UDP + beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan.
Pathway	Protein modification; protein glycosylation.
Subunit structure	Interacts with GALNT5 By similarity. Forms a homo/hetero-oligomeric complex with EXT1.
Subcellular location	Endoplasmic reticulum membrane; Single-pass type II membrane protein. Golgi apparatus membrane; Single-pass type II membrane protein. Note: The EXT1/EXT2 complex is localized in the Golgi apparatus.
Tissue specificity	Heart, brain, spleen, lung, liver, skeletal muscle, kidney and testis.
Developmental stage	Expressed in early stages of embryonic development.
Sequence similarities	Belongs to the glycosyltransferase 47 family.

Ontologies

Keywords
Cellular component	Endoplasmic reticulum Golgi apparatus Membrane
Domain	Signal-anchor Transmembrane
Molecular function	Glycosyltransferase Transferase
PTM	Glycoprotein
Gene Ontology (GO)
Biological process	cell differentiation Inferred from mutant phenotype. Source: MGI heparan sulfate proteoglycan biosynthetic process Inferred from mutant phenotype. Source: MGI mesoderm formation Inferred from mutant phenotype. Source: MGI
Cellular component	Golgi membrane Ref.4 Inferred by curator. Source: UniProtKB integral to membrane Inferred from electronic annotation. Source: UniProtKB-KW intrinsic to endoplasmic reticulum membrane Inferred from electronic annotation. Source: InterPro
Molecular function	N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity Ref.4 Inferred by curator. Source: UniProtKB glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity Ref.4 Inferred by curator. Source: UniProtKB heparan sulfate N-acetylglucosaminyltransferase activity Ref.4 Inferred by curator. Source: UniProtKB protein homodimerization activity Ref.4 Inferred from direct assay. Source: UniProtKB
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 718	718	Exostosin-2		PRO_0000149652
Regions
	Topological domain	1 – 25	25	Cytoplasmic Potential
	Transmembrane	26 – 46	21	Signal-anchor for type II membrane protein Potential
	Topological domain	47 – 718	672	Lumenal Potential
Amino acid modifications
	Glycosylation	288	1	N-linked (GlcNAc...) Potential
	Glycosylation	637	1	N-linked (GlcNAc...) Potential
Experimental info
	Sequence conflict	62	1	S → T in AAC53143. Ref.2
	Sequence conflict	160	1	L → V in AAC53143. Ref.2
	Sequence conflict	323	1	R → G in AAH06597. Ref.3
	Sequence conflict	355	1	V → I in AAC53143. Ref.2
	Sequence conflict	360	1	K → R in AAH06597. Ref.3
	Sequence conflict	389	1	Q → H in AAC53143. Ref.2

Sequences

	Sequence		Length	Mass (Da)
	P70428-1 [UniParc]. Last modified February 1, 1997. Version 1. Checksum: C2E765393A7F7ABD	FASTA	718	82,105
	10 20 30 40 50 60 MCASVKSNIR GPALIPRMKT KHRIYYVTLF SIVLLGLIAT GMFQFWPHSI ESSSDGGVEK 70 80 90 100 110 120 RSIREVPVVR LPTDSPIPER GDLSCRMHTC FDVYRCGFNP KNKIKVYIYP LKKYVDDAGV 130 140 150 160 170 180 PVSSAISREY NELLTAISDS DYYTDDINRA CLFVPSIDVL NQNPLRIKET AQALAQLSRW 190 200 210 220 230 240 DRGTNHLLFN MLPGAPPDYN TALDVPRDRA LLAGGGFSTW TYRQGYDVSI PVFSPLSAEM 250 260 270 280 290 300 ALPEKAPGPR RYFLLSSQMA IHPEYREELE ALQAKHQESV LVLDKCTNLS EGVLSVRKRC 310 320 330 340 350 360 HQHQVFDYPQ VLQEATFCTV LRRARLGQAV LSDVLQAGCV PVVIADSYIL PFSEVLDWKK 370 380 390 400 410 420 ASVVVPEEKM SDVYSILQNI PQRQIEEMQR QARWFWEAYF QSIKAIALAT LQIINDRIYP 430 440 450 460 470 480 YAAISYEEWN DPPAVKWASV SNPLFLPLIP PQSQGFTAIV LTYDRVESLF RVITEVSKVP 490 500 510 520 530 540 SLSKLLVVWN NQNKNPPEES LWPKIRVPLK VVRTAENKLS NRFFPYDEIE TEAVLAIDDD 550 560 570 580 590 600 IIMLTSDELQ FGYEVWREFP DRLVGYPGRL HLWDHEMNKW KYESEWTNEV SMVLTGAAFY 610 620 630 640 650 660 HKYFNYLYTY KMPGDIKNWV DAHMNCEDIA MNFLVANVTG KAVIKVTPRK KFKCPECTAI 670 680 690 700 710 DGLSLDQTHM VERSECINKF ASVFGTMPLK VVEHRADPVL YKDDFPEKLK SFPNIGSL

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Isolation and characterization of the murine homolog of the human EXT2 multiple exostoses gene." Stickens D.J., Evans G.A. Biochem. Mol. Med. 61:16-21(1997) [PubMed: 9232192] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[2]	"The structure of the human multiple exostoses 2 gene and characterization of homologs in mouse and Caenorhabditis elegans." Clines G.A., Ashley J.A., Shah S., Lovett M. Genome Res. 7:359-367(1997) [PubMed: 9110175] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. Tissue: Brain.
[3]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Mammary gland.
[4]	"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., Duncan G., Goutsos K.T., Tufaro F. Proc. Natl. Acad. Sci. U.S.A. 97:668-673(2000) [PubMed: 10639137] [Abstract] Cited for: CHARACTERIZATION.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBLGenBankDDBJ	U72141 mRNA. Translation: AAB17006.1. U67837 mRNA. Translation: AAC53143.1. BC006597 mRNA. Translation: AAH06597.1.
IPI	IPI00308075.
UniGene	Mm.4336
3D structure databases
HSSP	HSSP built from PDB template 1OMX based on UniProtKB Q9ES89.
ModBase	Search...
Protein family/group databases
CAZy	GT47. Glycosyltransferase Family 47. GT64. Glycosyltransferase Family 64.
Genome annotation databases
Ensembl	ENSMUSG00000027198. Mus musculus. [Contig view]
KEGG	mmu:14043.
Organism-specific databases
MGI	MGI:108050. Ext2.
Phylogenomic databases
HOVERGEN	P70428.
Enzyme and pathway databases
BRENDA	2.4.1.225. 244.
Gene expression databases
ArrayExpress	P70428.
Bgee	P70428.
CleanEx	MM_EXT2.
GermOnline	ENSMUSG00000027198. Mus musculus.
Family and domain databases
InterPro	IPR004263. Exostosin. IPR015338. HexNAc_Trfase_a. [Graphical view]
Pfam	PF03016. Exostosin. 1 hit. PF09258. Glyco_transf_64. 1 hit. [Graphical view]
ProtoNet	Search...
Other Resources
NextBio	284982.
SOURCE	Search...

Entry information

Entry name

EXT2_MOUSE

Accession

Primary (citable) accession number: P70428
Secondary accession number(s): P70395, Q923D6

Entry history

Integrated into UniProtKB/Swiss-Prot:	July 15, 1998
Last sequence update:	February 1, 1997
Last modified:	June 16, 2009
This is version 74 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)