Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Exostosin-1

Gene

Ext1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Required for the exosomal release of SDCBP, CD63 and syndecan (By similarity).By similarity

Catalytic activityi

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.

Cofactori

Mn2+By similarity

Pathwayi: protein glycosylation

This protein is involved in the pathway protein glycosylation, which is part of Protein modification.
View all proteins of this organism that are known to be involved in the pathway protein glycosylation and in Protein modification.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei518SubstrateBy similarity1
Metal bindingi567Manganese; catalyticBy similarity1
Binding sitei595SubstrateBy similarity1
Active sitei654By similarity1

GO - Molecular functioni

GO - Biological processi

  • axon guidance Source: MGI
  • brain development Source: MGI
  • cellular polysaccharide biosynthetic process Source: MGI
  • embryonic skeletal joint development Source: MGI
  • endoderm development Source: MGI
  • gastrulation Source: MGI
  • glycosaminoglycan biosynthetic process Source: MGI
  • heparan sulfate proteoglycan biosynthetic process Source: UniProtKB
  • heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process Source: MGI
  • mesoderm development Source: MGI
  • olfactory bulb development Source: MGI
  • ossification Source: MGI
  • protein glycosylation Source: UniProtKB-UniPathway
Complete GO annotation...

Keywords - Molecular functioni

Glycosyltransferase, Transferase

Keywords - Ligandi

Manganese, Metal-binding

Enzyme and pathway databases

BRENDAi2.4.1.225. 3474.
ReactomeiR-MMU-2022928. HS-GAG biosynthesis.
UniPathwayiUPA00378.

Protein family/group databases

CAZyiGT47. Glycosyltransferase Family 47.
GT64. Glycosyltransferase Family 64.

Names & Taxonomyi

Protein namesi
Recommended name:
Exostosin-1 (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 1 homolog
Gene namesi
Name:Ext1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 15

Organism-specific databases

MGIiMGI:894663. Ext1.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 7CytoplasmicSequence analysis7
Transmembranei8 – 28Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST21
Topological domaini29 – 746LumenalSequence analysisAdd BLAST718

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum, Golgi apparatus, Membrane

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001496491 – 746Exostosin-1Add BLAST746

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi89N-linked (GlcNAc...)Sequence analysis1
Glycosylationi330N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi652 ↔ 704By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

PaxDbiP97464.
PeptideAtlasiP97464.
PRIDEiP97464.

PTM databases

iPTMnetiP97464.
PhosphoSitePlusiP97464.

Expressioni

Tissue specificityi

Expressed in maturing chondrocytes.

Developmental stagei

Initially expressed at 6.5 dpc, which coincides with gastrulation of the embryo. High level of expression in developing limb buds at 10.5 to 12.5 dpc.1 Publication

Gene expression databases

BgeeiENSMUSG00000061731.
CleanExiMM_EXT1.
ExpressionAtlasiP97464. baseline and differential.
GenevisibleiP97464. MM.

Interactioni

Subunit structurei

Forms a homo/hetero-oligomeric complex with EXT2.By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000076505.

Structurei

3D structure databases

ProteinModelPortaliP97464.
SMRiP97464.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni544 – 549Substrate bindingBy similarity6
Regioni565 – 567Substrate bindingBy similarity3
Regioni650 – 654Substrate bindingBy similarity5
Regioni688 – 701Substrate bindingBy similarityAdd BLAST14

Sequence similaritiesi

Belongs to the glycosyltransferase 47 family.Curated

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1021. Eukaryota.
ENOG410XTFH. LUCA.
GeneTreeiENSGT00550000074496.
HOGENOMiHOG000266990.
HOVERGENiHBG003459.
InParanoidiP97464.
KOiK02366.
OMAiVTTCKHG.
OrthoDBiEOG091G0BDR.
PhylomeDBiP97464.
TreeFamiTF314231.

Family and domain databases

Gene3Di3.90.550.10. 1 hit.
InterProiIPR004263. Exostosin.
IPR027670. Exostosin-1.
IPR015338. EXT_C.
IPR029044. Nucleotide-diphossugar_trans.
[Graphical view]
PANTHERiPTHR11062:SF97. PTHR11062:SF97. 2 hits.
PfamiPF03016. Exostosin. 1 hit.
PF09258. Glyco_transf_64. 1 hit.
[Graphical view]
SUPFAMiSSF53448. SSF53448. 1 hit.

Sequencei

Sequence statusi: Complete.

P97464-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MQAKKRYFIL LSAGSCLALL FYFGGVQFRA SRSHSRREEH SGRNGLHQPS
60 70 80 90 100
PDHFWPRFPD ALRPFFPWDQ LENEDSSVHI SPRQKRDANS SIYKGKKCRM
110 120 130 140 150
ESCFDFTLCK KNGFKVYVYP QQKGEKIAES YQNILAAIEG SRFYTSDPSQ
160 170 180 190 200
ACLFVLSLDT LDRDQLSPQY VHNLRSKVQS LHLWNNGRNH LIFNLYSGTW
210 220 230 240 250
PDYTEDVGFD IGQAMLAKAS ISTENFRPNF DVSIPLFSKD HPRTGGERGF
260 270 280 290 300
LKFNTIPPLR KYMLVFKGKR YLTGIGSDTR NALYHVHNGE DVLLLTTCKH
310 320 330 340 350
GKDWQKHKDS RCDRDNTEYE KYDYREMLHN ATFCLVPRGR RLGSFRFLEA
360 370 380 390 400
LQAACVPVML SNGWELPFSE VINWNQAAVI GDERLLLQIP STIRSIHQDK
410 420 430 440 450
ILALRQQTQF LWEAYFSSVE KIVLTTLEII QDRIFKHISR NSLIWNKHPG
460 470 480 490 500
GLFVLPQYSS YLGDFPYYYA NLGLKPPSKF TAVIHAVTPL VSQSQPVLKL
510 520 530 540 550
LVAAAKSQYC AQIIVLWNCD KPLPAKHRWP ATAVPVIVIE GESKVMSSRF
560 570 580 590 600
LPYDNIITDA VLSLDEDTVL STTEVDFAFT VWQSFPERIV GYPARSHFWD
610 620 630 640 650
NSKERWGYTS KWTNDYSMVL TGAAIYHKYY HYLYSHYLPA SLKNMVDQLA
660 670 680 690 700
NCEDILMNFL VSAVTKLPPI KVTQKKQYKE TMMGQTSRAS RWADPDHFAQ
710 720 730 740
RQSCMNTFAS WFGYMPLIHS QMRLDPVLFK DQVSILRKKY RDIERL
Length:746
Mass (Da):86,308
Last modified:May 1, 1997 - v1
Checksum:i5AC7F24BECEEDFF9
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti16C → S in CAA65443 (PubMed:9254013).Curated1
Sequence conflicti60 – 61DA → EP in CAA65443 (PubMed:9254013).Curated2
Sequence conflicti220S → T in CAA65443 (PubMed:9254013).Curated1
Sequence conflicti486A → P in CAA65443 (PubMed:9254013).Curated1
Sequence conflicti548Missing in CAA65443 (PubMed:9254013).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U78539 mRNA. Translation: AAB41728.1.
X96639 mRNA. Translation: CAA65443.1.
BC004741 mRNA. Translation: AAH04741.1.
CCDSiCCDS27466.1.
RefSeqiNP_034292.2. NM_010162.2.
UniGeneiMm.309395.

Genome annotation databases

EnsembliENSMUST00000077273; ENSMUSP00000076505; ENSMUSG00000061731.
GeneIDi14042.
KEGGimmu:14042.
UCSCiuc007vrh.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U78539 mRNA. Translation: AAB41728.1.
X96639 mRNA. Translation: CAA65443.1.
BC004741 mRNA. Translation: AAH04741.1.
CCDSiCCDS27466.1.
RefSeqiNP_034292.2. NM_010162.2.
UniGeneiMm.309395.

3D structure databases

ProteinModelPortaliP97464.
SMRiP97464.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000076505.

Protein family/group databases

CAZyiGT47. Glycosyltransferase Family 47.
GT64. Glycosyltransferase Family 64.

PTM databases

iPTMnetiP97464.
PhosphoSitePlusiP97464.

Proteomic databases

PaxDbiP97464.
PeptideAtlasiP97464.
PRIDEiP97464.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000077273; ENSMUSP00000076505; ENSMUSG00000061731.
GeneIDi14042.
KEGGimmu:14042.
UCSCiuc007vrh.2. mouse.

Organism-specific databases

CTDi2131.
MGIiMGI:894663. Ext1.

Phylogenomic databases

eggNOGiKOG1021. Eukaryota.
ENOG410XTFH. LUCA.
GeneTreeiENSGT00550000074496.
HOGENOMiHOG000266990.
HOVERGENiHBG003459.
InParanoidiP97464.
KOiK02366.
OMAiVTTCKHG.
OrthoDBiEOG091G0BDR.
PhylomeDBiP97464.
TreeFamiTF314231.

Enzyme and pathway databases

UniPathwayiUPA00378.
BRENDAi2.4.1.225. 3474.
ReactomeiR-MMU-2022928. HS-GAG biosynthesis.

Miscellaneous databases

ChiTaRSiExt1. mouse.
PROiP97464.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000061731.
CleanExiMM_EXT1.
ExpressionAtlasiP97464. baseline and differential.
GenevisibleiP97464. MM.

Family and domain databases

Gene3Di3.90.550.10. 1 hit.
InterProiIPR004263. Exostosin.
IPR027670. Exostosin-1.
IPR015338. EXT_C.
IPR029044. Nucleotide-diphossugar_trans.
[Graphical view]
PANTHERiPTHR11062:SF97. PTHR11062:SF97. 2 hits.
PfamiPF03016. Exostosin. 1 hit.
PF09258. Glyco_transf_64. 1 hit.
[Graphical view]
SUPFAMiSSF53448. SSF53448. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiEXT1_MOUSE
AccessioniPrimary (citable) accession number: P97464
Secondary accession number(s): Q61546
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: May 1, 1997
Last modified: November 2, 2016
This is version 142 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.