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Protein

Xylosyltransferase 2

Gene

Xylt2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at transcript leveli

Functioni

Catalyzes the first step in the biosynthesis of chondroitin sulfate, heparan sulfate and dermatan sulfate proteoglycans, such as DCN (PubMed:17517600). Transfers D-xylose from UDP-D-xylose to specific serine residues of the core protein (By similarity).By similarity1 Publication

Catalytic activityi

UDP-alpha-D-xylose + [protein]-L-serine = UDP + [protein]-3-O-(beta-D-xylosyl)-L-serine.By similarity

Cofactori

Mg2+By similarity, Mn2+By similarityNote: Active with either Mg2+ or Mn2+, but activity is highest when both are present.By similarity

Pathwayi: chondroitin sulfate biosynthesis

This protein is involved in the pathway chondroitin sulfate biosynthesis, which is part of Glycan metabolism.By similarity
View all proteins of this organism that are known to be involved in the pathway chondroitin sulfate biosynthesis and in Glycan metabolism.

Pathwayi: heparan sulfate biosynthesis

This protein is involved in the pathway heparan sulfate biosynthesis, which is part of Glycan metabolism.By similarity
View all proteins of this organism that are known to be involved in the pathway heparan sulfate biosynthesis and in Glycan metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei239UDP-xylose; via carbonyl oxygenBy similarity1
Binding sitei267UDP-xyloseBy similarity1
Binding sitei481UDP-xyloseBy similarity1

GO - Molecular functioni

GO - Biological processi

  • chondroitin sulfate biosynthetic process Source: UniProtKB-UniPathway
  • chondroitin sulfate proteoglycan biosynthetic process Source: UniProtKB
  • glycosaminoglycan biosynthetic process Source: MGI
  • heparan sulfate proteoglycan biosynthetic process Source: UniProtKB
  • heparin biosynthetic process Source: MGI
  • proteoglycan biosynthetic process Source: MGI

Keywordsi

Molecular functionGlycosyltransferase, Transferase
LigandMagnesium, Manganese, Metal-binding

Enzyme and pathway databases

ReactomeiR-MMU-1971475 A tetrasaccharide linker sequence is required for GAG synthesis
UniPathwayiUPA00755
UPA00756

Protein family/group databases

CAZyiGT14 Glycosyltransferase Family 14

Names & Taxonomyi

Protein namesi
Recommended name:
Xylosyltransferase 2 (EC:2.4.2.26)
Alternative name(s):
Peptide O-xylosyltransferase 2
Xylosyltransferase II
Gene namesi
Name:Xylt2
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 11

Organism-specific databases

MGIiMGI:2444797 Xylt2

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 – 15CytoplasmicSequence analysisAdd BLAST15
Transmembranei16 – 36Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST21
Topological domaini37 – 865LumenalSequence analysisAdd BLAST829

Keywords - Cellular componenti

Golgi apparatus, Membrane, Secreted

Pathology & Biotechi

Disruption phenotypei

Mutant mice are born at the expected Mendelian rate. Their livers display strongly reduced levels of heparan sulfate proteoglycan. DCN glycosylation is altered and lacks chondroitin sulfate groups. After 3 to 5 months, all mutant mice display increased liver weight. At an age of 4 to 5 months, about half of them delevop liver cysts, due to biliary epithelial cell hyperplasia. At an age of 3 and 10 months, mutant mice also display increased kidney weight due to hydronephrosis and impaired renal function, but they do not develop cysts.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001914071 – 865Xylosyltransferase 2Add BLAST865

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi122N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi162 ↔ 190By similarity
Disulfide bondi206 ↔ 448By similarity
Glycosylationi327N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi467 ↔ 480By similarity
Disulfide bondi469 ↔ 478By similarity
Disulfide bondi581 ↔ 833By similarity
Glycosylationi683N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi826 ↔ 839By similarity

Post-translational modificationi

Contains disulfide bonds.By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

MaxQBiQ9EPL0
PaxDbiQ9EPL0
PRIDEiQ9EPL0

PTM databases

iPTMnetiQ9EPL0
PhosphoSitePlusiQ9EPL0

Expressioni

Tissue specificityi

Detected in brain, liver, lung, kidney, heart, spleen and testis, and at lower levels in skeletal muscle.1 Publication

Gene expression databases

BgeeiENSMUSG00000020868
CleanExiMM_XYLT2
ExpressionAtlasiQ9EPL0 baseline and differential
GenevisibleiQ9EPL0 MM

Interactioni

Subunit structurei

Monomer.By similarity

Protein-protein interaction databases

IntActiQ9EPL0, 1 interactor
STRINGi10090.ENSMUSP00000112052

Structurei

3D structure databases

ProteinModelPortaliQ9EPL0
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni296 – 298UDP-xylose bindingBy similarity3
Regioni400 – 401UDP-xylose bindingBy similarity2
Regioni504 – 505UDP-xylose bindingBy similarity2

Sequence similaritiesi

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0799 Eukaryota
ENOG410XQ7M LUCA
GeneTreeiENSGT00760000119183
HOGENOMiHOG000070086
HOVERGENiHBG059443
InParanoidiQ9EPL0
KOiK00771
OMAiWRMVTIW
OrthoDBiEOG091G020K
PhylomeDBiQ9EPL0
TreeFamiTF315534

Family and domain databases

InterProiView protein in InterPro
IPR003406 Glyco_trans_14
IPR024448 XylT
PfamiView protein in Pfam
PF02485 Branch, 1 hit
PF12529 Xylo_C, 1 hit

Sequencei

Sequence statusi: Complete.

Q9EPL0-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MVASARVQKL VRRYKLAIAT ALAILLLQGL VVWSFSGLEE DEPGEKGRQR
60 70 80 90 100
KPRPLDPGEG SKDTDSSAGR RGSAGRRHGR WRGRAESPGV PVAKVVRAVT
110 120 130 140 150
SRQRASRRVP PAPPPEAPGR QNLSGAAAGE ALIGAPGFPQ HGDTGSVEGA
160 170 180 190 200
PQPTDNTFTP KCEIVGKDAL SALARASTKQ CQQEIANVVC LHQAGNLMPK
210 220 230 240 250
SVPRHCQLAG KMSPGVQWEE IRAQQPVGGP PVRIAYMLVV HGRAIRQLKR
260 270 280 290 300
LLKAVYHEQH FFYIHVDKRS NYLYREVVEL AQHYENVRVT PWRMVTIWGG
310 320 330 340 350
ASLLRMYLRS MKDLLEIPGW TWDFFINLSA TDYPTRTNEE LVAFLSKNRD
360 370 380 390 400
KNFLKSHGRD NSRFIKKQGL DRLFHECDSH MWRLGERQIP AGIVVDGGSD
410 420 430 440 450
WFVLTRSFVE YVVYTDDPLV AQLRQFYTYT LLPAESFFHT VLENSPACAS
460 470 480 490 500
LVDNNLRVTN WNRKLGCKCQ YKHIVDWCGC SPNDFKPQDF LRLQQVSRPT
510 520 530 540 550
FFARKFESTV NQEVLEILDF HLYGSYPPST PALKAYWENI YDVADGPGGL
560 570 580 590 600
SDVLLTAYTA FARLSLRHAA TAVSPLATAV CRFEPRGLPS SVHLYFYDDH
610 620 630 640 650
FQGYLVTQAV QPSAQGPAET LEMWLMPQRS LKLLGHSDQA SRLQSLEVGT
660 670 680 690 700
EWDPKERLFR NFGGLLGPLD EPVAMQRWAR GPNLTATVVW IDPTYVVATS
710 720 730 740 750
YDITVDADTE VTQYKPPLSL PLRPGAWTVR LLQFWEPLGE TRFLVLPLTF
760 770 780 790 800
NRKLPLRKDD ASWLHAGPPH NEYMEQSFQG LSGILNLPQA EALEEAARRH
810 820 830 840 850
TELTGSALEA WTDGELSNFW SVAGLCAIGP SACPSLELCR LTSWSSLSPD
860
PKSELGPVKA DGRLR
Length:865
Mass (Da):96,811
Last modified:May 10, 2005 - v3
Checksum:iDA30B77B9807A797
GO

Sequence cautioni

The sequence AAH34082 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti94K → N in CAC18567 (Ref. 1) Curated1
Sequence conflicti460N → K in CAC18567 (Ref. 1) Curated1
Sequence conflicti466 – 467GC → AG in CAC18567 (Ref. 1) Curated2
Sequence conflicti478C → S in CAC18567 (Ref. 1) Curated1
Sequence conflicti483N → K in CAC18567 (Ref. 1) Curated1
Sequence conflicti487P → R in CAC18567 (Ref. 1) Curated1
Sequence conflicti504 – 506RKF → GKL in CAC18567 (Ref. 1) Curated3
Sequence conflicti515L → V in CAC18567 (Ref. 1) Curated1
Sequence conflicti666L → V in CAC18567 (Ref. 1) Curated1
Sequence conflicti672P → A in CAC18567 (Ref. 1) Curated1
Sequence conflicti675 – 676MQ → LR in CAC18567 (Ref. 1) Curated2
Sequence conflicti685T → R in CAC18567 (Ref. 1) Curated1
Sequence conflicti768 – 770PPH → QPQ in CAC18567 (Ref. 1) Curated3
Sequence conflicti780G → V in CAC18567 (Ref. 1) Curated1
Sequence conflicti788P → S in CAC18567 (Ref. 1) Curated1
Sequence conflicti799R → Q in CAC18567 (Ref. 1) Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ291751 mRNA Translation: CAC18567.2
AL645764 Genomic DNA No translation available.
BC034082 mRNA Translation: AAH34082.1 Different initiation.
CCDSiCCDS48888.1
RefSeqiNP_665827.2, NM_145828.3
UniGeneiMm.100638

Genome annotation databases

EnsembliENSMUST00000116349; ENSMUSP00000112052; ENSMUSG00000020868
GeneIDi217119
KEGGimmu:217119
UCSCiuc007kzi.2 mouse

Similar proteinsi

Entry informationi

Entry nameiXYLT2_MOUSE
AccessioniPrimary (citable) accession number: Q9EPL0
Secondary accession number(s): Q5SUY1, Q8K060
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 10, 2005
Last sequence update: May 10, 2005
Last modified: July 18, 2018
This is version 120 of the entry and version 3 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. 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

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