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Protein

Chondroitin sulfate synthase 3

Gene

CHSY3

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

Functioni

Has both beta-1,3-glucuronic acid and beta-1,4-N-acetylgalactosamine transferase activity. Transfers glucuronic acid (GlcUA) from UDP-GlcUA and N-acetylgalactosamine (GalNAc) from UDP-GalNAc to the non-reducing end of the elongating chondroitin polymer. Specific activity is much reduced compared to CHSY1.1 Publication

Catalytic activityi

UDP-N-acetyl-alpha-D-galactosamine + beta-D-glucuronosyl-(1->3)-N-acetyl-beta-D-galactosaminyl-proteoglycan = UDP + N-acetyl-beta-D-galactosaminyl-(1->4)-beta-D-glucuronosyl-(1->3)-N-acetyl-beta-D-galactosaminyl-proteoglycan.
UDP-alpha-D-glucuronate + N-acetyl-beta-D-galactosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan = UDP + beta-D-glucuronosyl-(1->3)-N-acetyl-beta-D-galactosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan.

Cofactori

Co2+1 Publication, Mn2+1 Publication, Cd2+1 PublicationNote: Divalent metal cations. Highest activities are measured with Co2+, Mn2+ and Cd2+.1 Publication

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi720 – 7201Divalent metal cationSequence analysis
Metal bindingi834 – 8341Divalent metal cationSequence analysis

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Ligandi

Metal-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS11959-MONOMER.
BRENDAi2.4.1.175. 2681.
ReactomeiR-HSA-2022870. Chondroitin sulfate biosynthesis.
SABIO-RKQ70JA7.

Protein family/group databases

CAZyiGT31. Glycosyltransferase Family 31.
GT7. Glycosyltransferase Family 7.

Names & Taxonomyi

Protein namesi
Recommended name:
Chondroitin sulfate synthase 3 (EC:2.4.1.175, EC:2.4.1.226)
Alternative name(s):
Carbohydrate synthase 2
Chondroitin glucuronyltransferase 3
Chondroitin synthase 2
Short name:
ChSy-2
Glucuronosyl-N-acetylgalactosaminyl-proteoglycan 4-beta-N-acetylgalactosaminyltransferase II
N-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase 3
N-acetylgalactosaminyltransferase 3
Gene namesi
Name:CHSY3
Synonyms:CHSY2, CSS3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

HGNCiHGNC:24293. CHSY3.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 77CytoplasmicSequence analysis
Transmembranei8 – 2821Helical; Signal-anchor for type II membrane proteinSequence analysisAdd
BLAST
Topological domaini29 – 882854LumenalSequence analysisAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA162382259.

Polymorphism and mutation databases

BioMutaiCHSY3.
DMDMi145559453.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 882882Chondroitin sulfate synthase 3PRO_0000189562Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi155 – 1551N-linked (GlcNAc...)Sequence analysis
Glycosylationi279 – 2791N-linked (GlcNAc...)Sequence analysis
Glycosylationi710 – 7101N-linked (GlcNAc...)Sequence analysis
Glycosylationi878 – 8781N-linked (GlcNAc...)Sequence analysis

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiQ70JA7.
MaxQBiQ70JA7.
PaxDbiQ70JA7.
PRIDEiQ70JA7.

PTM databases

iPTMnetiQ70JA7.
PhosphoSiteiQ70JA7.

Expressioni

Tissue specificityi

Detected at low levels in brain, cerebral cortex, uterus and small intestine.1 Publication

Gene expression databases

BgeeiQ70JA7.
CleanExiHS_CHSY3.
GenevisibleiQ70JA7. HS.

Organism-specific databases

HPAiHPA044612.

Interactioni

Protein-protein interaction databases

BioGridi130623. 15 interactions.
IntActiQ70JA7. 1 interaction.
STRINGi9606.ENSP00000302629.

Structurei

3D structure databases

ProteinModelPortaliQ70JA7.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Compositional bias

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Compositional biasi211 – 23525Pro-richAdd
BLAST
Compositional biasi539 – 5446Poly-Leu

Sequence similaritiesi

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3588. Eukaryota.
ENOG410XNYM. LUCA.
GeneTreeiENSGT00760000119143.
HOGENOMiHOG000220809.
HOVERGENiHBG050948.
InParanoidiQ70JA7.
KOiK13499.
OMAiDRVNCPR.
OrthoDBiEOG74TWXX.
PhylomeDBiQ70JA7.
TreeFamiTF318303.

Family and domain databases

Gene3Di3.90.550.10. 1 hit.
InterProiIPR008428. Chond_GalNAc.
IPR029044. Nucleotide-diphossugar_trans.
[Graphical view]
PfamiPF05679. CHGN. 1 hit.
[Graphical view]
SUPFAMiSSF53448. SSF53448. 3 hits.

Sequencei

Sequence statusi: Complete.

Q70JA7-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAVRSRRPWM SVALGLVLGF TAASWLIAPR VAELSERKRR GSSLCSYYGR
60 70 80 90 100
SAAGPRAGAQ QPLPQPQSRP RQEQSPPPAR QDLQGPPLPE AAPGITSFRS
110 120 130 140 150
SPWQQPPPLQ QRRRGREPEG ATGLPGAPAA EGEPEEEDGG AAGQRRDGRP
160 170 180 190 200
GSSHNGSGDG GAAAPSARPR DFLYVGVMTA QKYLGSRALA AQRTWARFIP
210 220 230 240 250
GRVEFFSSQQ PPNAGQPPPP LPVIALPGVD DSYPPQKKSF MMIKYMHDHY
260 270 280 290 300
LDKYEWFMRA DDDVYIKGDK LEEFLRSLNS SKPLYLGQTG LGNIEELGKL
310 320 330 340 350
GLEPGENFCM GGPGMIFSRE VLRRMVPHIG ECLREMYTTH EDVEVGRCVR
360 370 380 390 400
RFGGTQCVWS YEMQQLFHEN YEHNRKGYIQ DLHNSKIHAA ITLHPNKRPA
410 420 430 440 450
YQYRLHNYML SRKISELRYR TIQLHRESAL MSKLSNTEVS KEDQQLGVIP
460 470 480 490 500
SFNHFQPRER NEVIEWEFLT GKLLYSAAEN QPPRQSLSSI LRTALDDTVL
510 520 530 540 550
QVMEMINENA KSRGRLIDFK EIQYGYRRVN PMHGVEYILD LLLLYKRHKG
560 570 580 590 600
RKLTVPVRRH AYLQQLFSKP FFRETEELDV NSLVESINSE TQSFSFISNS
610 620 630 640 650
LKILSSFQGA KEMGGHNEKK VHILVPLIGR YDIFLRFMEN FENMCLIPKQ
660 670 680 690 700
NVKLVIILFS RDSGQDSSKH IELIKGYQNK YPKAEMTLIP MKGEFSRGLG
710 720 730 740 750
LEMASAQFDN DTLLLFCDVD LIFREDFLQR CRDNTIQGQQ VYYPIIFSQY
760 770 780 790 800
DPKVTNGGNP PTDDYFIFSK KTGFWRDYGY GITCIYKSDL LGAGGFDTSI
810 820 830 840 850
QGWGLEDVDL YNKVILSGLR PFRSQEVGVV HIFHPVHCDP NLDPKQYKMC
860 870 880
LGSKASTFAS TMQLAELWLE KHLGVRYNRT LS
Length:882
Mass (Da):100,284
Last modified:April 17, 2007 - v3
Checksum:i2FE26485B2CEA515
GO

Sequence cautioni

The sequence CAD43233.1 differs from that shown. Reason: Erroneous initiation. Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti173 – 1731L → P in CAE17326 (Ref. 2) Curated
Sequence conflicti195 – 1951W → R in CAE17326 (Ref. 2) Curated
Sequence conflicti202 – 2021R → H in CAE17326 (Ref. 2) Curated
Sequence conflicti268 – 2681G → E in CAE17326 (Ref. 2) Curated
Sequence conflicti337 – 3371Y → H in BAC98832 (PubMed:12907687).Curated
Sequence conflicti354 – 3552GT → RA in BAC98832 (PubMed:12907687).Curated
Sequence conflicti361 – 3622YE → FQ in BAC98832 (PubMed:12907687).Curated
Sequence conflicti856 – 8561S → N in CAD43233 (Ref. 2) Curated
Sequence conflicti856 – 8561S → N in CAE17326 (Ref. 2) Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti615 – 6151G → E.
Corresponds to variant rs10068403 [ dbSNP | Ensembl ].
VAR_027540
Natural varianti764 – 7641D → G.
Corresponds to variant rs2015018 [ dbSNP | Ensembl ].
VAR_021174

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB086062 mRNA. Translation: BAC98832.1.
AJ504664 mRNA. Translation: CAD43233.1. Different initiation.
AJ578034 mRNA. Translation: CAE17326.1.
BC137325 mRNA. Translation: AAI37326.1.
CCDSiCCDS34223.1.
RefSeqiNP_787052.3. NM_175856.4.
UniGeneiHs.213137.

Genome annotation databases

EnsembliENST00000305031; ENSP00000302629; ENSG00000198108.
GeneIDi337876.
KEGGihsa:337876.
UCSCiuc003kvd.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Web resourcesi

GGDB

GlycoGene database

Functional Glycomics Gateway - GTase

Chondroitin sulfate synthase 3

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB086062 mRNA. Translation: BAC98832.1.
AJ504664 mRNA. Translation: CAD43233.1. Different initiation.
AJ578034 mRNA. Translation: CAE17326.1.
BC137325 mRNA. Translation: AAI37326.1.
CCDSiCCDS34223.1.
RefSeqiNP_787052.3. NM_175856.4.
UniGeneiHs.213137.

3D structure databases

ProteinModelPortaliQ70JA7.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi130623. 15 interactions.
IntActiQ70JA7. 1 interaction.
STRINGi9606.ENSP00000302629.

Protein family/group databases

CAZyiGT31. Glycosyltransferase Family 31.
GT7. Glycosyltransferase Family 7.

PTM databases

iPTMnetiQ70JA7.
PhosphoSiteiQ70JA7.

Polymorphism and mutation databases

BioMutaiCHSY3.
DMDMi145559453.

Proteomic databases

EPDiQ70JA7.
MaxQBiQ70JA7.
PaxDbiQ70JA7.
PRIDEiQ70JA7.

Protocols and materials databases

DNASUi337876.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000305031; ENSP00000302629; ENSG00000198108.
GeneIDi337876.
KEGGihsa:337876.
UCSCiuc003kvd.4. human.

Organism-specific databases

CTDi337876.
GeneCardsiCHSY3.
HGNCiHGNC:24293. CHSY3.
HPAiHPA044612.
MIMi609963. gene.
neXtProtiNX_Q70JA7.
PharmGKBiPA162382259.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3588. Eukaryota.
ENOG410XNYM. LUCA.
GeneTreeiENSGT00760000119143.
HOGENOMiHOG000220809.
HOVERGENiHBG050948.
InParanoidiQ70JA7.
KOiK13499.
OMAiDRVNCPR.
OrthoDBiEOG74TWXX.
PhylomeDBiQ70JA7.
TreeFamiTF318303.

Enzyme and pathway databases

BioCyciMetaCyc:HS11959-MONOMER.
BRENDAi2.4.1.175. 2681.
ReactomeiR-HSA-2022870. Chondroitin sulfate biosynthesis.
SABIO-RKQ70JA7.

Miscellaneous databases

GenomeRNAii337876.
PROiQ70JA7.
SOURCEiSearch...

Gene expression databases

BgeeiQ70JA7.
CleanExiHS_CHSY3.
GenevisibleiQ70JA7. HS.

Family and domain databases

Gene3Di3.90.550.10. 1 hit.
InterProiIPR008428. Chond_GalNAc.
IPR029044. Nucleotide-diphossugar_trans.
[Graphical view]
PfamiPF05679. CHGN. 1 hit.
[Graphical view]
SUPFAMiSSF53448. SSF53448. 3 hits.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. Cited for: NUCLEOTIDE SEQUENCE [MRNA], FUNCTION, SUBCELLULAR LOCATION, COFACTOR, TISSUE SPECIFICITY.
  2. "A novel gene for chondroitin synthase in 5q23.3."
    Kamakari S., Chatzidakis I., Anagnou N.
    Submitted (FEB-2004) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Tissue: Pancreas and Placenta.
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].

Entry informationi

Entry nameiCHSS3_HUMAN
AccessioniPrimary (citable) accession number: Q70JA7
Secondary accession number(s): B2RP97, Q76L22, Q86Y52
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 2005
Last sequence update: April 17, 2007
Last modified: June 8, 2016
This is version 115 of the entry and version 3 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 5
    Human chromosome 5: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. 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.