Reactome | CHST14 transfers SO4(2-) to GalNAc in dermatan or DS

CHST14 transfers SO4(2-) to GalNAc in dermatan or DS

Stable Identifier

R-HSA-2022063

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

Synonyms

GalNAc is sulfated on position 4 of GalNAc in dermatan or dermatan sulfate

ReviewStatus

5/5

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CHST14 transfers SO4(2-) to GalNAc in dermatan or DS

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Important functional domains in dermatan or dermatan sulfate (DS) are generated by the action of an epimerase (which converts D-glucuronic acid into its epimer L-iduronic acid) together with 4-O-sulfation. These domains are named 4-O-sulfated iduronic acid blocks (Pachebo et al. 2009). Carbohydrate sulfotransferase 14 (CHST14) (Evers et al. 2001) mediates the transfer of sulfate to position 4 of another N-acetylgalactosamine (GalNAc) residue of D2,4(S)2-PG (sulfated on position 2 of IdoA and position 4 of GalNAc) to produce a further sulfated product D2,4,4(S)3-PG (sulfated on another GalNAc in addition to the ones above). Mutations in CHST14 can cause a form of Ehlers-Danlos syndrome (EDSMC1, MIM:601776; Miyake et al., 2010).

Literature References

PubMed ID	Title	Journal	Year
20533528	Loss-of-function mutations of CHST14 in a new type of Ehlers-Danlos syndrome Miyake, N, Kosho, T, Mizumoto, S, Furuichi, T, Hatamochi, A, Nagashima, Y, Arai, E, Takahashi, K, Kawamura, R, Wakui, K, Takahashi, J, Kato, H, Yasui, H, Ishida, T, Ohashi, H, Nishimura, G, Shiina, M, Saitsu, H, Tsurusaki, Y, Doi, H, Fukushima, Y, Ikegawa, S, Yamada, S, Sugahara, K, Matsumoto, N	Hum. Mutat.	2010
19661164	Dermatan 4-O-sulfotransferase 1 is pivotal in the formation of iduronic acid blocks in dermatan sulfate Pacheco, B, Maccarana, M, Malmström, A	Glycobiology	2009
11470797	Molecular cloning and characterization of a dermatan-specific N-acetylgalactosamine 4-O-sulfotransferase Evers, MR, Xia, G, Kang, HG, Schachner, M, Baenziger, JU	J. Biol. Chem.	2001