Reactome | CHST3,7 transfer SO4(2-) to position 6 of GalNAc on chondroitin chains

CHST3,7 transfer SO4(2-) to position 6 of GalNAc on chondroitin chains

Stable Identifier

R-HSA-2018682

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

Synonyms

Chondroitin can be sulfated on position 6 of GalNAc by CHST3,7

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CHST3,7 transfer SO4(2-) to position 6 of GalNAc on chondroitin chains

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Carbohydrate sulfotransferase 3 (CHST3 also known as C6ST-1) catalyzes the transfer of sulfate (SO4(2-)) from PAPS to position 6 of the N-acetylgalactosamine (GalNAc) residue of chondroitin-containing proteins resulting in chondroitin sulfate (C6S-PG), a major component of cartilage. Defects in CHST3 result in undersulfated CS, weakening cartilage structures and causing spondyloepiphyseal dysplasia with congenital joint dislocations (SEDC-JD; MIM:143095). Mutations causing SEDC-JD include R304Q, L259P, R222W, L307P, Y201*, E372K and L286P (Thiele et al. 2004, Hermanns et al. 2008, Unger et al. 2010, van Roij et al. 2008).

Literature References

PubMed ID	Title	Journal	Year
9883891	Functional expression and genomic structure of human chondroitin 6-sulfotransferase Tsutsumi, K, Shimakawa, H, Kitagawa, H, Sugahara, K	FEBS Lett	1998
10781596	Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase Kitagawa, H, Fujita, M, Ito, N, Sugahara, K	J Biol Chem	2000