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Protein

Glycosaminoglycan xylosylkinase

Gene

FAM20B

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Responsible for the 2-O-phosphorylation of xylose in the glycosaminoglycan-protein linkage region of proteoglycans thereby regulating the amount of mature GAG chains. Sulfated glycosaminoglycans (GAGs), including heparan sulfate and chondroitin sulfate, are synthesized on the so-called common GAG-protein linkage region (GlcUAbeta1-3Galbeta1-3Galbeta1-4Xylbeta1-O-Ser) of core proteins, which is formed by the stepwise addition of monosaccharide residues by the respective specific glycosyltransferases. Xylose 2-O-phosphorylation may influence the catalytic activity of B3GAT3 (GlcAT-I) which completes the precursor tetrasaccharide of GAG-protein linkage regions on which the repeating disaccharide region is synthesized.2 Publications

Catalytic activityi

ATP + D-xylose = ADP + D-xylose 2-phosphate.1 Publication

Cofactori

Mn2+By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei107ATP; via amide nitrogenBy similarity1
Binding sitei123ATPBy similarity1
Metal bindingi142ManganeseBy similarity1
Active sitei289By similarity1
Binding sitei294ATPBy similarity1
Metal bindingi309ManganeseBy similarity1
Binding sitei309ATPBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi222 – 225ATPBy similarity4

GO - Molecular functioni

GO - Biological processi

  • positive regulation of protein targeting to mitochondrion Source: ParkinsonsUK-UCL
Complete GO annotation...

Keywords - Molecular functioni

Kinase, Transferase

Keywords - Ligandi

ATP-binding, Manganese, Metal-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000116199-MONOMER.

Names & Taxonomyi

Protein namesi
Recommended name:
Glycosaminoglycan xylosylkinase (EC:2.7.1.-1 Publication)
Alternative name(s):
Xylose kinase
Gene namesi
Name:FAM20BImported
Synonyms:KIAA04751 Publication
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:23017. FAM20B.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 6CytoplasmicSequence analysis6
Transmembranei7 – 25Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST19
Topological domaini26 – 409LumenalSequence analysisAdd BLAST384

GO - Cellular componenti

  • Golgi apparatus Source: UniProtKB
  • Golgi membrane Source: UniProtKB-SubCell
  • integral component of membrane Source: UniProtKB-KW
  • nucleoplasm Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi9917.
OpenTargetsiENSG00000116199.
PharmGKBiPA134930918.

Polymorphism and mutation databases

BioMutaiFAM20B.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000087451 – 409Glycosaminoglycan xylosylkinaseAdd BLAST409

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi193N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi196 ↔ 211By similarity
Disulfide bondi201 ↔ 204By similarity
Disulfide bondi257 ↔ 331By similarity
Disulfide bondi332 ↔ 389By similarity

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

EPDiO75063.
MaxQBiO75063.
PaxDbiO75063.
PeptideAtlasiO75063.
PRIDEiO75063.

PTM databases

iPTMnetiO75063.
PhosphoSitePlusiO75063.

Expressioni

Tissue specificityi

Widely expressed. Strongly expressed in pancreas, spleen and fetal liver.2 Publications

Gene expression databases

BgeeiENSG00000116199.
CleanExiHS_FAM20B.
ExpressionAtlasiO75063. baseline and differential.
GenevisibleiO75063. HS.

Organism-specific databases

HPAiHPA007409.

Interactioni

Protein-protein interaction databases

BioGridi115245. 19 interactors.
IntActiO75063. 3 interactors.
STRINGi9606.ENSP00000263733.

Structurei

3D structure databases

ProteinModelPortaliO75063.
SMRiO75063.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the FAM20 family.Curated

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3829. Eukaryota.
ENOG410XQEJ. LUCA.
GeneTreeiENSGT00390000007484.
HOGENOMiHOG000231437.
HOVERGENiHBG051635.
InParanoidiO75063.
KOiK20825.
OMAiCTDQFGM.
OrthoDBiEOG091G1BEO.
PhylomeDBiO75063.
TreeFamiTF313276.

Family and domain databases

InterProiIPR024869. FAM20.
IPR009581. FAM20_C.
[Graphical view]
PANTHERiPTHR12450. PTHR12450. 1 hit.
PfamiPF06702. Fam20C. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

O75063-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKLKQRVVLL AILLVIFIFT KVFLIDNLDT SAANREDQRA FHRMMTGLRV
60 70 80 90 100
ELAPKLDHTL QSPWEIAAQW VVPREVYPEE TPELGAVMHA MATKKIIKAD
110 120 130 140 150
VGYKGTQLKA LLILEGGQKV VFKPKRYSRD HVVEGEPYAG YDRHNAEVAA
160 170 180 190 200
FHLDRILGFH RAPLVVGRFV NLRTEIKPVA TEQLLSTFLT VGNNTCFYGK
210 220 230 240 250
CYYCRETEPA CADGDIMEGS VTLWLPDVWP LQKHRHPWGR TYREGKLARW
260 270 280 290 300
EYDESYCDAV KKTSPYDSGP RLLDIIDTAV FDYLIGNADR HHYESFQDDE
310 320 330 340 350
GASMLILLDN AKSFGNPSLD ERSILAPLYQ CCIIRVSTWN RLNYLKNGVL
360 370 380 390 400
KSALKSAMAH DPISPVLSDP HLDAVDQRLL SVLATVKQCT DQFGMDTVLV

EDRMPLSHL
Length:409
Mass (Da):46,432
Last modified:November 1, 1998 - v1
Checksum:i03E211C0AF37B766
GO

Sequence cautioni

The sequence BAA32320 differs from that shown. Reason: Erroneous initiation.Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB480690 mRNA. Translation: BAH79819.1.
AB007944 mRNA. Translation: BAA32320.2. Different initiation.
AK289989 mRNA. Translation: BAF82678.1.
AL139132 Genomic DNA. Translation: CAH70923.1.
AL139132 Genomic DNA. Translation: CAH70924.1.
CH471067 Genomic DNA. Translation: EAW91034.1.
BC046441 mRNA. Translation: AAH46441.1.
BC051794 mRNA. Translation: AAH51794.1.
CCDSiCCDS1328.1.
RefSeqiNP_001311239.1. NM_001324310.1.
NP_001311240.1. NM_001324311.1.
NP_055679.1. NM_014864.3.
UniGeneiHs.5737.

Genome annotation databases

EnsembliENST00000263733; ENSP00000263733; ENSG00000116199.
GeneIDi9917.
KEGGihsa:9917.
UCSCiuc001gmc.4. human.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB480690 mRNA. Translation: BAH79819.1.
AB007944 mRNA. Translation: BAA32320.2. Different initiation.
AK289989 mRNA. Translation: BAF82678.1.
AL139132 Genomic DNA. Translation: CAH70923.1.
AL139132 Genomic DNA. Translation: CAH70924.1.
CH471067 Genomic DNA. Translation: EAW91034.1.
BC046441 mRNA. Translation: AAH46441.1.
BC051794 mRNA. Translation: AAH51794.1.
CCDSiCCDS1328.1.
RefSeqiNP_001311239.1. NM_001324310.1.
NP_001311240.1. NM_001324311.1.
NP_055679.1. NM_014864.3.
UniGeneiHs.5737.

3D structure databases

ProteinModelPortaliO75063.
SMRiO75063.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi115245. 19 interactors.
IntActiO75063. 3 interactors.
STRINGi9606.ENSP00000263733.

PTM databases

iPTMnetiO75063.
PhosphoSitePlusiO75063.

Polymorphism and mutation databases

BioMutaiFAM20B.

Proteomic databases

EPDiO75063.
MaxQBiO75063.
PaxDbiO75063.
PeptideAtlasiO75063.
PRIDEiO75063.

Protocols and materials databases

DNASUi9917.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000263733; ENSP00000263733; ENSG00000116199.
GeneIDi9917.
KEGGihsa:9917.
UCSCiuc001gmc.4. human.

Organism-specific databases

CTDi9917.
DisGeNETi9917.
GeneCardsiFAM20B.
HGNCiHGNC:23017. FAM20B.
HPAiHPA007409.
MIMi611063. gene.
neXtProtiNX_O75063.
OpenTargetsiENSG00000116199.
PharmGKBiPA134930918.
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3829. Eukaryota.
ENOG410XQEJ. LUCA.
GeneTreeiENSGT00390000007484.
HOGENOMiHOG000231437.
HOVERGENiHBG051635.
InParanoidiO75063.
KOiK20825.
OMAiCTDQFGM.
OrthoDBiEOG091G1BEO.
PhylomeDBiO75063.
TreeFamiTF313276.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000116199-MONOMER.

Miscellaneous databases

ChiTaRSiFAM20B. human.
GeneWikiiFAM20B.
GenomeRNAii9917.
PROiO75063.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000116199.
CleanExiHS_FAM20B.
ExpressionAtlasiO75063. baseline and differential.
GenevisibleiO75063. HS.

Family and domain databases

InterProiIPR024869. FAM20.
IPR009581. FAM20_C.
[Graphical view]
PANTHERiPTHR12450. PTHR12450. 1 hit.
PfamiPF06702. Fam20C. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiXYLK_HUMAN
AccessioniPrimary (citable) accession number: O75063
Secondary accession number(s): Q5W0C3, Q5W0C4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: September 19, 2003
Last sequence update: November 1, 1998
Last modified: November 30, 2016
This is version 124 of the entry and version 1 of the sequence. [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 1
    Human chromosome 1: 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

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.