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Protein

Xyloside xylosyltransferase 1

Gene

XXYLT1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Alpha-1,3-xylosyltransferase, which elongates the O-linked xylose-glucose disaccharide attached to EGF-like repeats in the extracellular domain of target proteins by catalyzing the addition of the second xylose (PubMed:22117070, PubMed:8982869). Known targets include Notch proteins and coagulation factors, such as F9 (PubMed:22117070, PubMed:8982869).2 Publications

Caution

It is uncertain whether Met-1 or Met-14 is the initiator.Curated

Catalytic activityi

UDP-alpha-D-xylose + alpha-D-xylose-(1->3)-beta-D-glucosyl-R = UDP + alpha-D-xylose-(1->3)-alpha-D-xylose-(1->3)-beta-D-glucosyl-R.2 Publications

Cofactori

Mg2+1 Publication, Mn2+1 PublicationNote: Has the highest in vitro activity with 20 mM Mn2+, a concentration entirely out of the physiological range. Can also utilize Mg2+, suggesting this may be the physiological cofactor.1 Publication

Kineticsi

  1. KM=0.28 mM for UDP-alpha-D-xylose1 Publication

    pH dependencei

    Optimum pH is 7.2.1 Publication

    Sites

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Metal bindingi226ManganeseBy similarity1
    Binding sitei227UDP-alpha-D-xylose; via amide nitrogenBy similarity1
    Metal bindingi228ManganeseBy similarity1
    Binding sitei290UDP-alpha-D-xyloseBy similarity1
    Binding sitei328UDP-alpha-D-xylose; via carbonyl oxygenBy similarity1
    Binding sitei331Substrate glycopeptideBy similarity1
    Binding sitei331UDP-alpha-D-xyloseBy similarity1
    Binding sitei360Substrate glycopeptideBy similarity1
    Metal bindingi383ManganeseBy similarity1
    Binding sitei385Substrate glycopeptideBy similarity1

    GO - Molecular functioni

    • magnesium ion binding Source: UniProtKB
    • manganese ion binding Source: UniProtKB
    • UDP-xylosyltransferase activity Source: UniProtKB

    GO - Biological processi

    • O-glycan processing Source: UniProtKB

    Keywordsi

    Molecular functionGlycosyltransferase, Transferase
    LigandMagnesium, Manganese, Metal-binding

    Enzyme and pathway databases

    SIGNORiQ8NBI6

    Protein family/group databases

    CAZyiGT8 Glycosyltransferase Family 8

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Xyloside xylosyltransferase 11 Publication (EC:2.4.2.n32 Publications)
    Alternative name(s):
    UDP-xylose:alpha-xyloside alpha-1,3-xylosyltransferase
    Gene namesi
    Name:XXYLT1
    Synonyms:C3orf21
    ORF Names:PSEC0251
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    Proteomesi
    • UP000005640 Componenti: Chromosome 3

    Organism-specific databases

    EuPathDBiHostDB:ENSG00000173950.15
    HGNCiHGNC:26639 XXYLT1
    MIMi614552 gene
    neXtProtiNX_Q8NBI6

    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 – 23Cytoplasmic1 PublicationAdd BLAST23
    Transmembranei24 – 44Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST21
    Topological domaini45 – 393Lumenal1 PublicationAdd BLAST349

    Keywords - Cellular componenti

    Endoplasmic reticulum, Membrane

    Pathology & Biotechi

    Organism-specific databases

    OpenTargetsiENSG00000173950
    PharmGKBiPA134925222

    Polymorphism and mutation databases

    BioMutaiXXYLT1
    DMDMi74751171

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    ChainiPRO_00002344271 – 393Xyloside xylosyltransferase 1Add BLAST393

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Disulfide bondi350 ↔ 375By similarity
    Disulfide bondi357 ↔ 386By similarity

    Keywords - PTMi

    Disulfide bond

    Proteomic databases

    EPDiQ8NBI6
    MaxQBiQ8NBI6
    PaxDbiQ8NBI6
    PeptideAtlasiQ8NBI6
    PRIDEiQ8NBI6
    ProteomicsDBi72773
    72774 [Q8NBI6-2]
    72775 [Q8NBI6-3]

    PTM databases

    iPTMnetiQ8NBI6
    PhosphoSitePlusiQ8NBI6

    Expressioni

    Gene expression databases

    BgeeiENSG00000173950
    CleanExiHS_C3orf21
    ExpressionAtlasiQ8NBI6 baseline and differential
    GenevisibleiQ8NBI6 HS

    Interactioni

    Subunit structurei

    Homodimer (PubMed:22117070). Dimer formation may be essential for the retention in endoplasmic reticulum (Probable).Curated1 Publication

    Protein-protein interaction databases

    BioGridi127415, 25 interactors
    STRINGi9606.ENSP00000309640

    Structurei

    3D structure databases

    ProteinModelPortaliQ8NBI6
    SMRiQ8NBI6
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Region

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Regioni104 – 106UDP-alpha-D-xylose bindingBy similarity3
    Regioni263 – 266Interaction with target proteinsBy similarity4

    Sequence similaritiesi

    Belongs to the glycosyltransferase 8 family.Curated

    Keywords - Domaini

    Signal-anchor, Transmembrane, Transmembrane helix

    Phylogenomic databases

    eggNOGiKOG3765 Eukaryota
    ENOG410XRNY LUCA
    GeneTreeiENSGT00530000063165
    HOGENOMiHOG000007690
    HOVERGENiHBG061240
    InParanoidiQ8NBI6
    OMAiCKVIFHD
    OrthoDBiEOG091G0GY6
    PhylomeDBiQ8NBI6
    TreeFamiTF323210

    Family and domain databases

    Gene3Di3.90.550.10, 1 hit
    InterProiView protein in InterPro
    IPR002495 Glyco_trans_8
    IPR029044 Nucleotide-diphossugar_trans
    PfamiView protein in Pfam
    PF01501 Glyco_transf_8, 1 hit
    SUPFAMiSSF53448 SSF53448, 1 hit

    Sequences (3)i

    Sequence statusi: Complete.

    This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

    Isoform 1 (identifier: Q8NBI6-1) [UniParc]FASTAAdd to basket

    This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

    « Hide

            10         20         30         40         50
    MGLLRGGLPC ARAMARLGAV RSHYCALLLA AALAVCAFYY LGSGRETFSS
    60 70 80 90 100
    ATKRLKEARA GAPAAPSPPA LELARGSVAP APGAKAKSLE GGGAGPVDYH
    110 120 130 140 150
    LLMMFTKAEH NAALQAKARV ALRSLLRLAK FEAHEVLNLH FVSEEASREV
    160 170 180 190 200
    AKGLLRELLP PAAGFKCKVI FHDVAVLTDK LFPIVEAMQK HFSAGLGTYY
    210 220 230 240 250
    SDSIFFLSVA MHQIMPKEIL QIIQLDLDLK FKTNIRELFE EFDSFLPGAI
    260 270 280 290 300
    IGIAREMQPV YRHTFWQFRH ENPQTRVGGP PPEGLPGFNS GVMLLNLEAM
    310 320 330 340 350
    RQSPLYSRLL EPAQVQQLAD KYHFRGHLGD QDFFTMIGME HPKLFHVLDC
    360 370 380 390
    TWNRQLCTWW RDHGYSDVFE AYFRCEGHVK IYHGNCNTPI PED
    Length:393
    Mass (Da):43,807
    Last modified:October 1, 2002 - v1
    Checksum:i3C788AFDF933A23D
    GO
    Isoform 2 (identifier: Q8NBI6-2) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1-203: Missing.
         204-217: IFFLSVAMHQIMPK → MWPPRCKDPGRQSA

    Note: No experimental confirmation available.
    Show »
    Length:190
    Mass (Da):22,304
    Checksum:i8E76B9CC7AFB4FD5
    GO
    Isoform 3 (identifier: Q8NBI6-3) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1-203: Missing.
         204-206: Missing.
         207-262: LSVAMHQIMP...IAREMQPVYR → MNGMSVLMKE...WSWTSQPLEL

    Note: No experimental confirmation available.
    Show »
    Length:187
    Mass (Da):21,817
    Checksum:i9A49152D460573D2
    GO

    Sequence cautioni

    The sequence AAH19036 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

    Alternative sequence

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Alternative sequenceiVSP_0183151 – 203Missing in isoform 2 and isoform 3. 1 PublicationAdd BLAST203
    Alternative sequenceiVSP_018317204 – 217IFFLS…QIMPK → MWPPRCKDPGRQSA in isoform 2. 1 PublicationAdd BLAST14
    Alternative sequenceiVSP_018316204 – 206Missing in isoform 3. 1 Publication3
    Alternative sequenceiVSP_018318207 – 262LSVAM…QPVYR → MNGMSVLMKETLKSSVTPSP YEETGERQPSVNWAVSPHQT QNQWARWSWTSQPLEL in isoform 3. 1 PublicationAdd BLAST56

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    AK057046 mRNA Translation: BAB71355.1
    AK092474 mRNA Translation: BAC03899.1
    AK075551 mRNA Translation: BAC11694.1
    CH471052 Genomic DNA Translation: EAW78028.1
    CH471052 Genomic DNA Translation: EAW78029.1
    BC019036 mRNA Translation: AAH19036.1 Different initiation.
    BC039067 mRNA Translation: AAH39067.2
    CCDSiCCDS43188.1 [Q8NBI6-1]
    RefSeqiNP_689744.3, NM_152531.4 [Q8NBI6-1]
    XP_016861239.1, XM_017005750.1 [Q8NBI6-2]
    UniGeneiHs.478741
    Hs.744847

    Genome annotation databases

    EnsembliENST00000310380; ENSP00000309640; ENSG00000173950 [Q8NBI6-1]
    ENST00000356740; ENSP00000349179; ENSG00000173950 [Q8NBI6-3]
    ENST00000437101; ENSP00000409865; ENSG00000173950 [Q8NBI6-2]
    GeneIDi152002
    KEGGihsa:152002
    UCSCiuc003fuk.4 human [Q8NBI6-1]

    Keywords - Coding sequence diversityi

    Alternative splicing

    Similar proteinsi

    Entry informationi

    Entry nameiXXLT1_HUMAN
    AccessioniPrimary (citable) accession number: Q8NBI6
    Secondary accession number(s): D3DNW5
    , Q8NAL3, Q8WV03, Q96ME0
    Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 16, 2006
    Last sequence update: October 1, 2002
    Last modified: June 20, 2018
    This is version 112 of the entry and version 1 of the sequence. See complete history.
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. Human chromosome 3
      Human chromosome 3: entries, gene names and cross-references to MIM
    2. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    3. SIMILARITY comments
      Index of protein domains and families

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