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Protein

Carbohydrate sulfotransferase 3

Gene

CHST3

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

Functioni

Sulfotransferase that utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as sulfonate donor to catalyze the transfer of sulfate to position 6 of the N-acetylgalactosamine (GalNAc) residue of chondroitin. Chondroitin sulfate constitutes the predominant proteoglycan present in cartilage and is distributed on the surfaces of many cells and extracellular matrices. Can also sulfate Gal residues of keratan sulfate, another glycosaminoglycan, and the Gal residues in sialyl N-acetyllactosamine (sialyl LacNAc) oligosaccharides. May play a role in the maintenance of naive T-lymphocytes in the spleen.2 Publications

Catalytic activityi

3'-phosphoadenylyl sulfate + chondroitin = adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate.

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi141 – 147PAPSBy similarity7
Nucleotide bindingi301 – 309PAPSBy similarity9

GO - Molecular functioni

  • chondroitin 6-sulfotransferase activity Source: UniProtKB
  • N-acetylglucosamine 6-O-sulfotransferase activity Source: GO_Central
  • sulfotransferase activity Source: ProtInc

GO - Biological processi

  • carbohydrate metabolic process Source: UniProtKB-KW
  • chondroitin sulfate biosynthetic process Source: UniProtKB
  • sulfur compound metabolic process Source: UniProtKB

Keywordsi

Molecular functionTransferase
Biological processCarbohydrate metabolism

Enzyme and pathway databases

BioCyciMetaCyc:HS04610-MONOMER
BRENDAi2.8.2.17 2681
ReactomeiR-HSA-2022870 Chondroitin sulfate biosynthesis
R-HSA-3595172 Defective CHST3 causes SEDCJD

Names & Taxonomyi

Protein namesi
Recommended name:
Carbohydrate sulfotransferase 3 (EC:2.8.2.17)
Alternative name(s):
Chondroitin 6-O-sulfotransferase 1
Short name:
C6ST-1
Chondroitin 6-sulfotransferase
Galactose/N-acetylglucosamine/N-acetylglucosamine 6-O-sulfotransferase 0
Short name:
GST-0
Gene namesi
Name:CHST3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 10

Organism-specific databases

EuPathDBiHostDB:ENSG00000122863.5
HGNCiHGNC:1971 CHST3
MIMi603799 gene
neXtProtiNX_Q7LGC8

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 – 20CytoplasmicSequence analysisAdd BLAST20
Transmembranei21 – 38Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST18
Topological domaini39 – 479LumenalSequence analysisAdd BLAST441

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Involvement in diseasei

Spondyloepiphyseal dysplasia with congenital joint dislocations (SEDCJD)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA bone dysplasia clinically characterized by dislocation of the knees and/or hips at birth, clubfoot, elbow joint dysplasia with subluxation and limited extension, short stature, and progressive kyphosis developing in late childhood. The disorder is usually evident at birth, with short stature and multiple joint dislocations or subluxations that dominate the neonatal clinical and radiographic picture. During childhood, the dislocations improve, both spontaneously and with surgical treatment, and features of spondyloepiphyseal dysplasia become apparent, leading to arthritis of the hips and spine with intervertebral disk degeneration, rigid kyphoscoliosis, and trunk shortening by late childhood.
See also OMIM:143095
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_047856222R → W in SEDCJD; severely impairs or abolishes the enzyme function. 1 PublicationCorresponds to variant dbSNP:rs121908617Ensembl.1
Natural variantiVAR_047857259L → P in SEDCJD; severely impairs or abolishes the enzyme function. 1 PublicationCorresponds to variant dbSNP:rs121908616Ensembl.1
Natural variantiVAR_021413304R → Q in SEDCJD; abolishes enzyme activity. 1 PublicationCorresponds to variant dbSNP:rs28937593Ensembl.1
Natural variantiVAR_047858307L → P in SEDCJD. 1 PublicationCorresponds to variant dbSNP:rs121908618Ensembl.1
Natural variantiVAR_047859372E → K in SEDCJD. 1 PublicationCorresponds to variant dbSNP:rs267606734Ensembl.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi9469
GeneReviewsiCHST3
MalaCardsiCHST3
MIMi143095 phenotype
OpenTargetsiENSG00000122863
Orphaneti263463 CHST3-related skeletal dysplasia
PharmGKBiPA26503

Polymorphism and mutation databases

BioMutaiCHST3
DMDMi116241297

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000851881 – 479Carbohydrate sulfotransferase 3Add BLAST479

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi63N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi74N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi96N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi256N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi420N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi464N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiQ7LGC8
MaxQBiQ7LGC8
PaxDbiQ7LGC8
PeptideAtlasiQ7LGC8
PRIDEiQ7LGC8

PTM databases

iPTMnetiQ7LGC8
PhosphoSitePlusiQ7LGC8

Expressioni

Tissue specificityi

Widely expressed in adult tissues. Expressed in heart, placenta, skeletal muscle and pancreas. Also expressed in various immune tissues such as spleen, lymph node, thymus and appendix.1 Publication

Gene expression databases

BgeeiENSG00000122863
CleanExiHS_CHST3
GenevisibleiQ7LGC8 HS

Organism-specific databases

HPAiHPA047523
HPA055704

Interactioni

Protein-protein interaction databases

BioGridi114855, 6 interactors
IntActiQ7LGC8, 1 interactor
STRINGi9606.ENSP00000362207

Structurei

3D structure databases

ProteinModelPortaliQ7LGC8
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiENOG410IIAV Eukaryota
ENOG410YDA4 LUCA
GeneTreeiENSGT00530000062902
HOGENOMiHOG000261614
HOVERGENiHBG106811
InParanoidiQ7LGC8
KOiK01020
OMAiFERYHCK
OrthoDBiEOG091G0V3Y
PhylomeDBiQ7LGC8
TreeFamiTF342871

Family and domain databases

InterProiView protein in InterPro
IPR016469 Carbohydrate_sulfotransferase
IPR027417 P-loop_NTPase
IPR000863 Sulfotransferase_dom
PfamiView protein in Pfam
PF00685 Sulfotransfer_1, 1 hit
PIRSFiPIRSF005883 Carbohydrate_sulfotransferase, 1 hit
SUPFAMiSSF52540 SSF52540, 2 hits

Sequencei

Sequence statusi: Complete.

Q7LGC8-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MEKGLTLPQD CRDFVHSLKM RSKYALFLVF VVIVFVFIEK ENKIISRVSD
60 70 80 90 100
KLKQIPQALA DANSTDPALI LAENASLLSL SELDSAFSQL QSRLRNLSLQ
110 120 130 140 150
LGVEPAMEAA GEEEEEQRKE EEPPRPAVAG PRRHVLLMAT TRTGSSFVGE
160 170 180 190 200
FFNQQGNIFY LFEPLWHIER TVSFEPGGAN AAGSALVYRD VLKQLFLCDL
210 220 230 240 250
YVLEHFITPL PEDHLTQFMF RRGSSRSLCE DPVCTPFVKK VFEKYHCKNR
260 270 280 290 300
RCGPLNVTLA AEACRRKEHM ALKAVRIRQL EFLQPLAEDP RLDLRVIQLV
310 320 330 340 350
RDPRAVLASR MVAFAGKYKT WKKWLDDEGQ DGLREEEVQR LRGNCESIRL
360 370 380 390 400
SAELGLRQPA WLRGRYMLVR YEDVARGPLQ KAREMYRFAG IPLTPQVEDW
410 420 430 440 450
IQKNTQAAHD GSGIYSTQKN SSEQFEKWRF SMPFKLAQVV QAACGPAMRL
460 470
FGYKLARDAA ALTNRSVSLL EERGTFWVT
Length:479
Mass (Da):54,706
Last modified:October 17, 2006 - v3
Checksum:i7C290DC4970F66E0
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti387R → P in BAA32576 (PubMed:9714738).Curated1
Sequence conflicti443A → P in BAA32576 (PubMed:9714738).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_047856222R → W in SEDCJD; severely impairs or abolishes the enzyme function. 1 PublicationCorresponds to variant dbSNP:rs121908617Ensembl.1
Natural variantiVAR_047857259L → P in SEDCJD; severely impairs or abolishes the enzyme function. 1 PublicationCorresponds to variant dbSNP:rs121908616Ensembl.1
Natural variantiVAR_021413304R → Q in SEDCJD; abolishes enzyme activity. 1 PublicationCorresponds to variant dbSNP:rs28937593Ensembl.1
Natural variantiVAR_047858307L → P in SEDCJD. 1 PublicationCorresponds to variant dbSNP:rs121908618Ensembl.1
Natural variantiVAR_021414348I → M. Corresponds to variant dbSNP:rs3740128Ensembl.1
Natural variantiVAR_021415357R → Q2 PublicationsCorresponds to variant dbSNP:rs3740129Ensembl.1
Natural variantiVAR_047859372E → K in SEDCJD. 1 PublicationCorresponds to variant dbSNP:rs267606734Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB012192 mRNA Translation: BAA32576.1
AB017915 mRNA Translation: BAA36348.1
BC093690 mRNA Translation: AAH93690.1
BC104856 mRNA Translation: AAI04857.1
CCDSiCCDS7312.1
RefSeqiNP_004264.2, NM_004273.4
XP_006718138.1, XM_006718075.3
XP_011538671.1, XM_011540369.2
UniGeneiHs.158304

Genome annotation databases

EnsembliENST00000373115; ENSP00000362207; ENSG00000122863
GeneIDi9469
KEGGihsa:9469
UCSCiuc001jsn.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiCHST3_HUMAN
AccessioniPrimary (citable) accession number: Q7LGC8
Secondary accession number(s): O75099, Q52M30
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 15, 2005
Last sequence update: October 17, 2006
Last modified: April 25, 2018
This is version 135 of the entry and version 3 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