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CHST3 carbohydrate (chondroitin 6) sulfotransferase 3 [ Homo sapiens (human) ]

Gene ID: 9469, updated on 13-Jan-2015
Official Symbol
CHST3provided by HGNC
Official Full Name
carbohydrate (chondroitin 6) sulfotransferase 3provided by HGNC
Primary source
HGNC:HGNC:1971
See related
Ensembl:ENSG00000122863; HPRD:07052; MIM:603799; Vega:OTTHUMG00000018431
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
HSD; C6ST; C6ST1
Summary
This gene encodes an enzyme which catalyzes the sulfation of chondroitin, a proteoglycan found in the extracellular matrix and most cells which is involved in cell migration and differentiation. Mutations in this gene are associated with spondylepiphyseal dysplasia and humerospinal dysostosis. [provided by RefSeq, Mar 2009]
Orthologs
See CHST3 in MapViewer
Location:
10q22.1
Exon count:
4
Annotation release Status Assembly Chr Location
106 current GRCh38 (GCF_000001405.26) 10 NC_000010.11 (71964362..72013564)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 10 NC_000010.10 (73724120..73773322)

Chromosome 10 - NC_000010.11Genomic Context describing neighboring genes Neighboring gene cadherin-related 23 Neighboring gene microRNA 7152 Neighboring gene RNA, U7 small nuclear 38 pseudogene Neighboring gene prosaposin Neighboring gene sparc/osteonectin, cwcv and kazal-like domains proteoglycan (testican) 2 Neighboring gene activating signal cointegrator 1 complex subunit 1 Neighboring gene ribosomal protein L15 pseudogene 14

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

Protein interactions

Protein Gene Interaction Pubs
Envelope surface glycoprotein gp160, precursor env Sulfate is linked to the carbohydrate chains of HIV-1 glycoproteins gp160, gp120, and gp41 during the endoplasmic reticulum to Golgi transport of the glycoproteins PubMed

Go to the HIV-1, Human Interaction Database

  • Chondroitin sulfate biosynthesis, organism-specific biosystem (from REACTOME)
    Chondroitin sulfate biosynthesis, organism-specific biosystemChondroitin sulfate (CS) glycosaminoglycan consists of N-acetylgalactosamine (GalNAc) residues alternating in glycosidic linkages with glucuronic acid (GlcA). GalNAc residues are sulfated to varying ...
  • Chondroitin sulfate/dermatan sulfate metabolism, organism-specific biosystem (from REACTOME)
    Chondroitin sulfate/dermatan sulfate metabolism, organism-specific biosystemChondroitin sulfate (CS) is a sulfated glycosaminoglycan (GAG). CS chains are unbranched polysaccharides of varying length containing two alternating monosaccharides: D-glucuronic acid (GlcA) and N-a...
  • Defective B3GAT3 causes JDSSDHD, organism-specific biosystem (from REACTOME)
    Defective B3GAT3 causes JDSSDHD, organism-specific biosystemGalactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferases1, 2 and 3 (B3GAT1-3) are involved in forming the linker tetrasaccharide present in heparan sulfate and chondroitin sulfate. Defects ...
  • Defective B4GALT1 causes B4GALT1-CDG (CDG-2d), organism-specific biosystem (from REACTOME)
    Defective B4GALT1 causes B4GALT1-CDG (CDG-2d), organism-specific biosystemCongenital disorders of glycosylation (CDG, previously called carbohydrate-deficient glycoprotein syndromes, CDGSs), are a group of hereditary multisystem disorders. They are characterized biochemica...
  • Defective B4GALT7 causes EDS, progeroid type, organism-specific biosystem (from REACTOME)
    Defective B4GALT7 causes EDS, progeroid type, organism-specific biosystemEhlersDanlos syndrome (EDS) is a group of inherited connective tissue disorders, caused by a defect in the synthesis of collagen types I or III. Abnormal collagen renders connective tissues more elas...
  • Defective CHST14 causes EDS, musculocontractural type, organism-specific biosystem (from REACTOME)
    Defective CHST14 causes EDS, musculocontractural type, organism-specific biosystemCarbohydrate sulfotransferase 14 (CHST14 also known as D4ST-1) mediates the transfer of sulfate to position 4 of further N-acetylgalactosamine (GalNAc) residues of dermatan sulfate (DS). Defects in C...
  • Defective CHST3 causes SEDCJD, organism-specific biosystem (from REACTOME)
    Defective CHST3 causes SEDCJD, organism-specific biosystemCarbohydrate sulfotransferase 3 (CHST3) transfers sulfate (SO4(2-)) to position 6 of N-acetylgalactosamine (GalNAc) residues of chondroitin-containg proteins resulting in chondroitin sulfate (CS), th...
  • Defective CHST6 causes MCDC1, organism-specific biosystem (from REACTOME)
    Defective CHST6 causes MCDC1, organism-specific biosystemCarbohydrate sulfotransferase 6 (CHST6) catalyzes the transfer of sulfate to position 6 of non-reducing ends of N-acetylglucosamine (GlcNAc) residues on keratan sulfate (KS). KS plays a central role ...
  • Defective CHSY1 causes TPBS, organism-specific biosystem (from REACTOME)
    Defective CHSY1 causes TPBS, organism-specific biosystemChondroitin sulfate synthases (CHSY) are involved in the synthesis of chondroitin sulfate, adding alternatingly glucuronate (GlcA) and N-acetylgalactosamine (GalNAc) to the growing chondroitin polyme...
  • Defective EXT1 causes exostoses 1, TRPS2 and CHDS, organism-specific biosystem (from REACTOME)
    Defective EXT1 causes exostoses 1, TRPS2 and CHDS, organism-specific biosystemHeparan sulfate (HS) is involved in regulating various body functions functions during development, homeostasis and pathology including blood clotting, angiogenesis and metastasis of cancer cells. Ex...
  • Defective EXT2 causes exostoses 2, organism-specific biosystem (from REACTOME)
    Defective EXT2 causes exostoses 2, organism-specific biosystemHeparan sulfate (HS) is involved in regulating various body functions during development, homeostasis and pathology including blood clotting, angiogenesis and metastasis of cancer cells. Exostosin 1 ...
  • Defective PAPSS2 causes SEMD-PA, organism-specific biosystem (from REACTOME)
    Defective PAPSS2 causes SEMD-PA, organism-specific biosystemDefects in PAPSS2 cause spondyloepimetaphyseal dysplasia Pakistani type (SEMD-PA; MIM:612847), a bone disease characterized by epiphyseal dysplasia with mild metaphyseal abnormalities. Clinical featu...
  • Defective SLC26A2 causes chondrodysplasias, organism-specific biosystem (from REACTOME)
    Defective SLC26A2 causes chondrodysplasias, organism-specific biosystemThe SLC26A1 and 2 genes encode sulfate transporter proteins that facilitate sulfate uptake into cells, critical in cartilage for sulfation of proteoglycans and extracellular matrix organization. Defe...
  • Disease, organism-specific biosystem (from REACTOME)
    Disease, organism-specific biosystemBiological processes are captured in Reactome by identifying the molecules (DNA, RNA, protein, small molecules) involved in them and describing the details of their interactions. From this molecular ...
  • Diseases associated with glycosaminoglycan metabolism, organism-specific biosystem (from REACTOME)
    Diseases associated with glycosaminoglycan metabolism, organism-specific biosystemA number of genetic disorders are caused by mutations in the genes encoding glycosyltransferases and sulfotransferases, enzymes responsible for the synthesis of glycosaminoglycans (GAGs) as well as ...
  • Diseases of glycosylation, organism-specific biosystem (from REACTOME)
    Diseases of glycosylation, organism-specific biosystemDiseases of glycosylation, usually referred to as congenital disorders of glycosylation (CDG), are rare inherited disorders ascribing defects of nucleotide-sugar biosynthesis and transport, glycosylt...
  • Glycogen storage diseases, organism-specific biosystem (from REACTOME)
    Glycogen storage diseases, organism-specific biosystemThe regulated turnover of glycogen plays a central, tissue-specific role in the maintenance of blood glucose levels and in the provision of glucose to tissues such as muscle and brain in response to ...
  • Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate, organism-specific biosystem (from KEGG)
    Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate, organism-specific biosystemGlycosaminoglycans (GAGs) are linear polysaccharide chains consisting of repeating disaccharide units and form proteglycans by covalently attaching to their core proteins. Chondroitin sulfate (CS) is...
  • Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate, conserved biosystem (from KEGG)
    Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate, conserved biosystemGlycosaminoglycans (GAGs) are linear polysaccharide chains consisting of repeating disaccharide units and form proteglycans by covalently attaching to their core proteins. Chondroitin sulfate (CS) is...
  • Glycosaminoglycan metabolism, organism-specific biosystem (from REACTOME)
    Glycosaminoglycan metabolism, organism-specific biosystemGlycosaminoglycans (GAGs) are long, unbranched polysaccharides containing a repeating disaccharide unit composed of a hexosamine (either N-acetylgalactosamine (GalNAc) or N-acetylglucosamine (GlcNAc)...
  • MPS I - Hurler syndrome, organism-specific biosystem (from REACTOME)
    MPS I - Hurler syndrome, organism-specific biosystemMucopolysaccharidosis type I (MPS I, Hurler syndrome, Hurler's disease, gargoylism, Scheie, Hirler-Scheie syndrome; MIM:607014, 607015 and 607016) is an autosomal recessive genetic disorder where th...
  • MPS II - Hunter syndrome, organism-specific biosystem (from REACTOME)
    MPS II - Hunter syndrome, organism-specific biosystemMucopolysaccharidosis II (MPS II, Hunter syndrome, MIM:309900) is an X-linked, recessive genetic disorder which therefore primarily affects males. MPS II was first described in 1917, by Major Charles...
  • MPS IIIA - Sanfilippo syndrome A, organism-specific biosystem (from REACTOME)
    MPS IIIA - Sanfilippo syndrome A, organism-specific biosystemMucopolysaccharidosis III (MPS III, Sanfilippo syndrome) was described in 1963 by a pediatrician named Sylvester Sanfilippo (J. Pediat. 63: 837-838, 1963, no reference). Mucopolysaccharidosis IIIA (M...
  • MPS IIIB - Sanfilippo syndrome B, organism-specific biosystem (from REACTOME)
    MPS IIIB - Sanfilippo syndrome B, organism-specific biosystemMucopolysaccharidosis III (Sanfilippo syndrome) was described in 1963 by a pediatrician named Sylvester Sanfilippo (J. Pediat. 63: 837838, 1963, no reference). MPS IIIB (Mucopolysaccharidosis type II...
  • MPS IIIC - Sanfilippo syndrome C, organism-specific biosystem (from REACTOME)
    MPS IIIC - Sanfilippo syndrome C, organism-specific biosystemMucopolysaccharidosis III (Sanfilippo syndrome) was described in 1963 by a pediatrician named Sylvester Sanfilippo (J. Pediat. 63: 837838, 1963, no reference). Mucopolysaccharidosis type IIIC (MPS II...
  • MPS IIID - Sanfilippo syndrome D, organism-specific biosystem (from REACTOME)
    MPS IIID - Sanfilippo syndrome D, organism-specific biosystemMucopolysaccharidosis III (Sanfilippo syndrome) was described in 1963 by a pediatrician named Sylvester Sanfilippo (J. Pediat. 63: 837-838, 1963, no reference). Mucopolysaccharidosis type IIID (MPS I...
  • MPS IV - Morquio syndrome A, organism-specific biosystem (from REACTOME)
    MPS IV - Morquio syndrome A, organism-specific biosystemMucopolysaccharidosis IV A (MPS IVA, MPS4A, Morquio's syndrome, Morquio's; MIM:253000) is a rare, autosomal recessive mucopolysaccharide storage disease, first described simultaneously in 1929 by L M...
  • MPS IV - Morquio syndrome B, organism-specific biosystem (from REACTOME)
    MPS IV - Morquio syndrome B, organism-specific biosystemDefects in beta-galactosidase (GLB1; MIM:611458) can result in GM1 gangliosidosis (GM1; MIM:230500) (Nishimoto et al. 1991) (not described here), with several phenotypes indicating mental deteriorati...
  • MPS IX - Natowicz syndrome, organism-specific biosystem (from REACTOME)
    MPS IX - Natowicz syndrome, organism-specific biosystemMucopolysaccharidosis type IX (MPS IX, Natowicz syndrome, Hyaluronidase deficiency, MIM:601492) is a rare lysosomal storage disease characterized by high hyaluronan (HA) concentration in the serum re...
  • MPS VI - Maroteaux-Lamy syndrome, organism-specific biosystem (from REACTOME)
    MPS VI - Maroteaux-Lamy syndrome, organism-specific biosystemMucopolysaccharidosis type VI (MPS VI, Maroteaux-Lamy syndrome, polydystrophic dwarfism; MIM:253200) is an autosomal recessive lysosomal storage disorder caused by a deficiency in arylsulfatase B (AR...
  • MPS VII - Sly syndrome, organism-specific biosystem (from REACTOME)
    MPS VII - Sly syndrome, organism-specific biosystemMucopolysaccharidosis type VII (MPS VII, Sly syndrome, beta-glucuronidase deficiency; MIM:253220) is an autosomal recessive lysosomal storage disease characterized by a deficiency of the enzyme beta-...
  • Metabolism, organism-specific biosystem (from REACTOME)
    Metabolism, organism-specific biosystemMetabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as th...
  • Metabolism of carbohydrates, organism-specific biosystem (from REACTOME)
    Metabolism of carbohydrates, organism-specific biosystemThese pathways together are responsible for: 1) the extraction of energy and carbon skeletons for biosyntheses from dietary sugars and related molecules; 2) the short-term storage of glucose in the b...
  • Mucopolysaccharidoses, organism-specific biosystem (from REACTOME)
    Mucopolysaccharidoses, organism-specific biosystemThe mucopolysaccharidoses (MPS) are a group of rare, inherited lysosomal storage disorders caused by deficiencies of enzymes catalyzing the stepwise degradation of glycosaminoglycans (GAGs, originall...
  • Myoclonic epilepsy of Lafora, organism-specific biosystem (from REACTOME)
    Myoclonic epilepsy of Lafora, organism-specific biosystemLafora disease is a progressive neurodegenerative disorder with onset typically late in childhood, characterized by seizures and progressive neurological deterioration and death within ten years of o...
  • chondroitin sulfate biosynthesis, organism-specific biosystem (from BIOCYC)
    chondroitin sulfate biosynthesis, organism-specific biosystem
    chondroitin sulfate biosynthesis
  • chondroitin sulfate biosynthesis, conserved biosystem (from BIOCYC)
    chondroitin sulfate biosynthesis, conserved biosystemGeneral Background |FRAME: Chondroitin-sulfates "Chondroitin sulfate"| is a sulfated |FRAME: Glycosaminoglycans glycosaminoglycan"| composed of alternating units of sulfated |FRAME:CPD-12557| and |F...
  • chondroitin sulfate biosynthesis (late stages), organism-specific biosystem (from BIOCYC)
    chondroitin sulfate biosynthesis (late stages), organism-specific biosystemGeneral Background : Chondroitin-sulfates "Chondroitin sulfate" is a sulfated : Glycosaminoglycans glycosaminoglycan" composed of alternating units of sulfated :CPD-12557 N-acetyl-beta-D-galactosami...
  • chondroitin sulfate biosynthesis (late stages), conserved biosystem (from BIOCYC)
    chondroitin sulfate biosynthesis (late stages), conserved biosystemGeneral Background |FRAME: Chondroitin-sulfates "Chondroitin sulfate"| is a sulfated |FRAME: Glycosaminoglycans glycosaminoglycan"| composed of alternating units of sulfated |FRAME:CPD-12557| and |F...
  • metapathway biotransformation, organism-specific biosystem (from WikiPathways)
    metapathway biotransformation, organism-specific biosystem
    metapathway biotransformation
Products Interactant Other Gene Complex Source Pubs Description

Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
chondroitin 6-sulfotransferase activity IDA
Inferred from Direct Assay
more info
PubMed 
proteoglycan sulfotransferase activity IEA
Inferred from Electronic Annotation
more info
 
sulfotransferase activity TAS
Traceable Author Statement
more info
PubMed 
Process Evidence Code Pubs
T cell homeostasis IEA
Inferred from Electronic Annotation
more info
 
carbohydrate metabolic process TAS
Traceable Author Statement
more info
 
chondroitin sulfate biosynthetic process IDA
Inferred from Direct Assay
more info
PubMed 
chondroitin sulfate biosynthetic process TAS
Traceable Author Statement
more info
 
chondroitin sulfate metabolic process TAS
Traceable Author Statement
more info
 
glycosaminoglycan metabolic process TAS
Traceable Author Statement
more info
 
pathogenesis TAS
Traceable Author Statement
more info
 
small molecule metabolic process TAS
Traceable Author Statement
more info
 
sulfur compound metabolic process IDA
Inferred from Direct Assay
more info
PubMed 
Component Evidence Code Pubs
Golgi membrane TAS
Traceable Author Statement
more info
 
integral component of membrane IEA
Inferred from Electronic Annotation
more info
 
Preferred Names
carbohydrate sulfotransferase 3
Names
carbohydrate sulfotransferase 3
GST-0
C6ST-1
chondroitin 6-O-sulfotransferase 1
galactose/N-acetylglucosamine/N-acetylglucosamine 6-O-sulfotransferase 0
NP_004264.2

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_012635.1 

    Range
    5001..54203
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_004273.4NP_004264.2  carbohydrate sulfotransferase 3

    See proteins identical to NP_004264.2

    Status: REVIEWED

    Source sequence(s)
    AB017915, AC022392, AI041015
    Consensus CDS
    CCDS7312.1
    UniProtKB/Swiss-Prot
    Q7LGC8
    Related
    ENSP00000362207, OTTHUMP00000019779, ENST00000373115, OTTHUMT00000048563
    Conserved Domains (1) summary
    pfam00685
    Location:269452
    Sulfotransfer_1; Sulfotransferase domain

RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 106

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38 Primary Assembly

Genomic

  1. NC_000010.11 

    Range
    71964362..72013564
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_006718075.1XP_006718138.1  

    See proteins identical to XP_006718138.1

    UniProtKB/Swiss-Prot
    Q7LGC8
    Conserved Domains (1) summary
    pfam00685
    Location:269452
    Sulfotransfer_1; Sulfotransferase domain

Alternate CHM1_1.1

Genomic

  1. NC_018921.2 

    Range
    74006249..74055464
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

Alternate HuRef

Genomic

  1. AC_000142.1 

    Range
    67718863..67768083
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)