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Protein

Glycosaminoglycan xylosylkinase

Gene

FAM20B

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

3-O-(beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl)-L-seryl-[protein] + ATP = 3-O-(beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-2-O-phosphoxylosyl)-L-seryl-[protein] + ADP.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

Keywordsi

Molecular functionKinase, Transferase
LigandATP-binding, Manganese, Metal-binding, Nucleotide-binding

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

EuPathDBiHostDB:ENSG00000116199.11
HGNCiHGNC:23017 FAM20B
MIMi611063 gene
neXtProtiNX_O75063

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 – 6CytoplasmicSequence analysis6
Transmembranei7 – 25Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST19
Topological domaini26 – 409LumenalSequence analysisAdd BLAST384

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...) asparagineSequence 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
ProteomicsDBi49734

PTM databases

iPTMnetiO75063
PhosphoSitePlusiO75063

Expressioni

Tissue specificityi

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

Gene expression databases

BgeeiENSG00000116199 Expressed in 242 organ(s), highest expression level in metanephric glomerulus
CleanExiHS_FAM20B
ExpressionAtlasiO75063 baseline and differential
GenevisibleiO75063 HS

Organism-specific databases

HPAiHPA007409

Interactioni

Binary interactionsi

WithEntry#Exp.IntActNotes
DCNP075854EBI-11090967,EBI-9663608

Protein-protein interaction databases

BioGridi115245, 26 interactors
IntActiO75063, 15 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
OMAiAFHRMMA
OrthoDBiEOG091G1BEO
PhylomeDBiO75063
TreeFamiTF313276

Family and domain databases

InterProiView protein in InterPro
IPR024869 FAM20
IPR009581 FAM20_C
PANTHERiPTHR12450 PTHR12450, 1 hit
PfamiView protein in Pfam
PF06702 Fam20C, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.iShow all

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

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
X6RH03X6RH03_HUMAN
Glycosaminoglycan xylosylkinase
FAM20B
138Annotation score:

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 No translation available.
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

Similar proteinsi

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 No translation available.
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, 26 interactors
IntActiO75063, 15 interactors
STRINGi9606.ENSP00000263733

PTM databases

iPTMnetiO75063
PhosphoSitePlusiO75063

Polymorphism and mutation databases

BioMutaiFAM20B

Proteomic databases

EPDiO75063
MaxQBiO75063
PaxDbiO75063
PeptideAtlasiO75063
PRIDEiO75063
ProteomicsDBi49734

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
EuPathDBiHostDB:ENSG00000116199.11
GeneCardsiFAM20B
HGNCiHGNC:23017 FAM20B
HPAiHPA007409
MIMi611063 gene
neXtProtiNX_O75063
OpenTargetsiENSG00000116199
PharmGKBiPA134930918
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

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

Miscellaneous databases

ChiTaRSiFAM20B human
GeneWikiiFAM20B
GenomeRNAii9917
PROiPR:O75063
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000116199 Expressed in 242 organ(s), highest expression level in metanephric glomerulus
CleanExiHS_FAM20B
ExpressionAtlasiO75063 baseline and differential
GenevisibleiO75063 HS

Family and domain databases

InterProiView protein in InterPro
IPR024869 FAM20
IPR009581 FAM20_C
PANTHERiPTHR12450 PTHR12450, 1 hit
PfamiView protein in Pfam
PF06702 Fam20C, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiXYLK_HUMAN
AccessioniPrimary (citable) accession number: O75063
Secondary accession number(s): Q5W0C3, Q5W0C4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 19, 2003
Last sequence update: November 1, 1998
Last modified: September 12, 2018
This is version 136 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
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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