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Protein

Exostosin-1

Gene

Ext1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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

Keywordsi

Molecular functionGlycosyltransferase, Transferase
LigandManganese, Metal-binding

Enzyme and pathway databases

BRENDAi2.4.1.225 3474
ReactomeiR-MMU-2022928 HS-GAG biosynthesis
UniPathwayi
UPA00378

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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 15

Organism-specific databases

MGIiMGI:894663 Ext1

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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

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...) asparagineSequence analysis1
Glycosylationi330N-linked (GlcNAc...) asparagineSequence 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 Expressed in 329 organ(s), highest expression level in cumulus cell
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
OMAiSHYWDDS
OrthoDBiEOG091G0BDR
PhylomeDBiP97464
TreeFamiTF314231

Family and domain databases

Gene3Di3.90.550.10, 1 hit
InterProiView protein in InterPro
IPR004263 Exostosin
IPR027670 Exostosin-1
IPR015338 EXT_C
IPR029044 Nucleotide-diphossugar_trans
PANTHERiPTHR11062 PTHR11062, 1 hit
PTHR11062:SF97 PTHR11062:SF97, 1 hit
PfamiView protein in Pfam
PF03016 Exostosin, 1 hit
PF09258 Glyco_transf_64, 1 hit
SUPFAMiSSF53448 SSF53448, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
D6RE56D6RE56_MOUSE
Exostosin-1
Ext1
44Annotation score:

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

Similar proteinsi

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
OMAiSHYWDDS
OrthoDBiEOG091G0BDR
PhylomeDBiP97464
TreeFamiTF314231

Enzyme and pathway databases

UniPathwayi
UPA00378

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

Miscellaneous databases

ChiTaRSiExt1 mouse
PROiPR:P97464
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000061731 Expressed in 329 organ(s), highest expression level in cumulus cell
CleanExiMM_EXT1
ExpressionAtlasiP97464 baseline and differential
GenevisibleiP97464 MM

Family and domain databases

Gene3Di3.90.550.10, 1 hit
InterProiView protein in InterPro
IPR004263 Exostosin
IPR027670 Exostosin-1
IPR015338 EXT_C
IPR029044 Nucleotide-diphossugar_trans
PANTHERiPTHR11062 PTHR11062, 1 hit
PTHR11062:SF97 PTHR11062:SF97, 1 hit
PfamiView protein in Pfam
PF03016 Exostosin, 1 hit
PF09258 Glyco_transf_64, 1 hit
SUPFAMiSSF53448 SSF53448, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiEXT1_MOUSE
AccessioniPrimary (citable) accession number: P97464
Secondary accession number(s): Q61546
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: May 1, 1997
Last modified: November 7, 2018
This is version 150 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

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

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