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

RefSeq Accession Definition Service Stock Status Price *Turnaround time Order
NM_004273 Homo sapiens carbohydrate (chondroitin 6) sulfotransferase 3 (CHST3), mRNA. GenEZ ORF Cloning On-demand $699.00 18
XM_006718075 PREDICTED: Homo sapiens carbohydrate (chondroitin 6) sulfotransferase 3 (CHST3), transcript variant X1, mRNA. GenEZ ORF Cloning On-demand $699.00 18

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Gene Symbol CHST3
Entrez Gene ID 9469
Full Name carbohydrate (chondroitin 6) sulfotransferase 3
Synonyms C6ST, C6ST1, HSD
General protein information
Preferred Names
carbohydrate sulfotransferase 3
carbohydrate sulfotransferase 3
chondroitin 6-O-sulfotransferase 1
galactose/N-acetylglucosamine/N-acetylglucosamine 6-O-sulfotransferase 0
Gene Type protein-coding
Organism Homo sapiens (human)



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].

MIM: 603799

Spondyloepiphyseal dysplasia with congenital joint dislocations,

mRNA Protein Product Sequence Price Select
NM_004273, 224493825 NP_004264, 19923281 carbohydrate sulfotransferase 3 ORF Sequence $550.00
XM_006718075, 578820025 XP_006718138, 578820026 carbohydrate sulfotransferase 3 isoform X1 ORF Sequence $550.00
hsa00532Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate
WP702metapathway biotransformation
HUMAN_PWY-6569chondroitin sulfate biosynthesis
META_PWY-6567chondroitin sulfate biosynthesis (late stages)
HUMAN_PWY-6567chondroitin sulfate biosynthesis (late stages)
META_PWY-6569chondroitin sulfate biosynthesis
REACT_474Metabolism of carbohydrates
REACT_147749MPS IIID - Sanfilippo syndrome D
REACT_147825MPS IV - Morquio syndrome A
REACT_147719MPS VI - Maroteaux-Lamy syndrome
REACT_147753MPS IIIA - Sanfilippo syndrome A
REACT_147739MPS IX - Natowicz syndrome
REACT_147860MPS IIIC - Sanfilippo syndrome C
REACT_147734MPS II - Hunter syndrome
REACT_147788MPS IIIB - Sanfilippo syndrome B
REACT_147759MPS VII - Sly syndrome
REACT_147798MPS IV - Morquio syndrome B
REACT_147857MPS I - Hurler syndrome
REACT_121315Glycosaminoglycan metabolism
REACT_120989Chondroitin sulfate biosynthesis
REACT_121206Chondroitin sulfate/dermatan sulfate metabolism
REACT_200783Myoclonic epilepsy of Lafora
REACT_200833Glycogen storage diseases
Homo sapiens (human)CHST3NP_004264.2
Pan troglodytes (chimpanzee)CHST3XP_521690.1
Macaca mulatta (Rhesus monkey)CHST3XP_001106464.2
Canis lupus familiaris (dog)CHST3XP_003639041.1
Bos taurus (cattle)CHST3NP_001192524.1
Mus musculus (house mouse)Chst3NP_058083.2
Rattus norvegicus (Norway rat)Chst3NP_445860.1
Gallus gallus (chicken)CHST3NP_990452.1
Danio rerio (zebrafish)chst3bNP_001103846.1
Danio rerio (zebrafish)chst3aNP_001139043.1
Xenopus (Silurana) tropicalis (western clawed frog)chst3XP_002939563.2
GO:0005975carbohydrate metabolic processTAS
GO:0006790sulfur compound metabolic processIDA
GO:0030203glycosaminoglycan metabolic processTAS
GO:0030204chondroitin sulfate metabolic processTAS
GO:0030206chondroitin sulfate biosynthetic processIDA
GO:0030206chondroitin sulfate biosynthetic processTAS
GO:0043029T cell homeostasisIEA
GO:0044281small molecule metabolic processTAS
GO:0000139Golgi membraneTAS
GO:0016021integral component of membraneIEA
GO:0008146sulfotransferase activityTAS
GO:0008459chondroitin 6-sulfotransferase activityIDA
GO:0050698proteoglycan sulfotransferase activityIEA
GeneCards CHST3
UniProt Q7LGC8
Vega OTTHUMG00000018431
MIM 603799
Ensembl ENSG00000122863
HGNC 1971
HPRD 07052

GeneRIFs: Gene References Into Functions What's a GeneRIF?

What is the normal function of the CHST3 gene?

The CHST3 gene provides instructions for making an enzyme called chondroitin 6-O-sulfotransferase 1 or C6ST-1. This enzyme has an important role in the development and maintenance of the skeleton. In particular, it is essential for the normal development of cartilage, which is a tough, flexible tissue that makes up much of the skeleton during early development. Most cartilage is later converted to bone, except for the cartilage that continues to cover and protect the ends of bones and is present in the nose and external ears.

The C6ST-1 enzyme modifies molecules called chondroitin sulfate proteoglycans, which are abundant in cartilage and give this tissue its rubbery, gel-like consistency. The C6ST-1 enzyme carries out a process known as sulfation, in which a chemical group called a sulfate is transferred from one chemical compound to another. Specifically, the enzyme takes sulfate from a molecule called 3'-phosphoadenyl-5'-phosphosulfate (PAPS) and adds it to a specific location on chondroitin sulfate proteoglycans. Sulfation of these molecules is a critical step in cartilage formation.


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