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
Synonyms
Chondroitin can be sulfated on position 6 of GalNAc by CHST3,7
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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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
chondroitin 6-sulfotransferase activity of CHST3,7 [Golgi membrane]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created