Reactome | CHPF,CHPF2,CHSY3 transfer GlcA to chondroitin

CHPF,CHPF2,CHSY3 transfer GlcA to chondroitin

Stable Identifier

R-HSA-1971491

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

Synonyms

A glucuronate moiety is added to chondroitin

ReviewStatus

5/5

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CHPF,CHPF2,CHSY3 transfer GlcA to chondroitin

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A glucuronate (GlcA) moiety is added to the chondroitin chain by dual-activity enzymes, the chondroitin sulfate synthases 1-3 (CHSY1, CHPF and CHSY3) (Kitagawa et al. 2001, Yada et al. 2003, Yada et al. 2003b). They possess both beta-1,3-glucuronic acid and beta-1,4-N-acetylgalactosamine transferase activity. These three enzymes require divalent metals as cofactors, manganese producing the highest activities. Another candidate enzyme, chondroitin sulfate glucuronyltransferase (CHPF2) possess only beta-1,3-glucuronic acid transferase activity (Izumikawa et al. 2008, Gotoh et al. 2002). Defects in CHSY1 cause Temtamy preaxial brachydactyly syndrome (TPBS) (MIM:605282) (Tian et al. 2010, Li et al. 2010).

Literature References

PubMed ID	Title	Journal	Year
21129728	Temtamy preaxial brachydactyly syndrome is caused by loss-of-function mutations in chondroitin synthase 1, a potential target of BMP signaling Yigit, G, Aglan, M, Laue, K, Baessmann, I, Wakeling, EL, Topaloglu, AK, Temtamy, S, Hammerschmidt, M, Kotan, LD, Amr, K, Nürnberg, G, Pawlik, B, Hegele, RA, Wollnik, B, Canan, H, Lanktree, MB, Li, Y, Nürnberg, P, Quarrell, OW, May, KW, Yilmaz, M	Am J Hum Genet	2010
11514575	Molecular cloning and expression of a human chondroitin synthase Uyama, T, Kitagawa, H, Sugahara, K	J Biol Chem	2001
12761225	Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities Yada, T, Kwon, YD, Kudo, T, Iwasaki, H, Gotoh, M, Watanabe, H, Go, M, Togayachi, A, Kimata, K, Sato, T, Kaseyama, H, Shionyu, M, Narimatsu, H, Kikuchi, N	J Biol Chem	2003
12145278	Molecular cloning and characterization of a novel chondroitin sulfate glucuronyltransferase that transfers glucuronic acid to N-acetylgalactosamine Inaba, N, Yada, T, Sato, T, Kudo, T, Iwasaki, H, Gotoh, M, Mochizuki, H, Watanabe, H, Narimatsu, H, Togayachi, A, Akashima, T, Kimata, K	J Biol Chem	2002
21129727	Loss of CHSY1, a secreted FRINGE enzyme, causes syndromic brachydactyly in humans via increased NOTCH signaling Ling, L, Lee, H, Al-Hadidy, A, Ababneh, OH, Hamamy, H, O'Connor, B, Merriman, B, Tian, J, Shboul, M, Masri, A, Nelson, SF, Reversade, B, Cool, S	Am J Hum Genet	2010
12907687	Chondroitin sulfate synthase-3. Molecular cloning and characterization Yada, T, Kwon, YD, Sato, T, Kudo, T, Iwasaki, H, Gotoh, M, Watanabe, H, Kaseyama, H, Narimatsu, H, Togayachi, A, Kikuchi, N, Kimata, K	J Biol Chem	2003
18316376	Identification of chondroitin sulfate glucuronyltransferase as chondroitin synthase-3 involved in chondroitin polymerization: chondroitin polymerization is achieved by multiple enzyme complexes consisting of chondroitin synthase family members Tamura, J, Shiozawa, S, Kitagawa, H, Sugahara, K, Izumikawa, T, Koike, T	J Biol Chem	2008