Reactome | Chondroitin polymerase complex transfers GalNAc to CS chain

Chondroitin polymerase complex transfers GalNAc to CS chain

Stable Identifier

R-HSA-1971487

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

Synonyms

GalNAc is added to chondroitin

ReviewStatus

5/5

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Chondroitin polymerase complex transfers GalNAc to CS chain

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The chondroitin polymerase complex transfers one GalNAc moiety from UDP-GalNAc to the CS chain. It consists of a heterodimer of CHSY1 or CHSY3 with CHPF2 or CHPF (Dutta et al., 2025; see also the review by Mikami & Kitagawa, 2013). They possess both beta-1,3-glucuronic acid and beta-1,4-N-acetylgalactosamine transferase activity (Kitagawa et al. 2001, Yada et al. 2003, Yada et al. 2003b). 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 Li, Y, Laue, K, Temtamy, S, Aglan, M, Kotan, LD, Yigit, G, Canan, H, Pawlik, B, Nürnberg, G, Wakeling, EL, Quarrell, OW, Baessmann, I, Lanktree, MB, Yilmaz, M, Hegele, RA, Amr, K, May, KW, Nürnberg, P, Topaloglu, AK, Hammerschmidt, M, Wollnik, B	Am J Hum Genet	2010
11514575	Molecular cloning and expression of a human chondroitin synthase Kitagawa, H, Uyama, T, Sugahara, K	J Biol Chem	2001
	Structural basis for human chondroitin sulfate chain polymerization Dutta, P
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, Gotoh, M, Sato, T, Shionyu, M, Go, M, Kaseyama, H, Iwasaki, H, Kikuchi, N, Kwon, YD, Togayachi, A, Kudo, T, Watanabe, H, Narimatsu, H, Kimata, K	J Biol Chem	2003
21129727	Loss of CHSY1, a secreted FRINGE enzyme, causes syndromic brachydactyly in humans via increased NOTCH signaling Tian, J, Ling, L, Shboul, M, Lee, H, O'Connor, B, Merriman, B, Nelson, SF, Cool, S, Ababneh, OH, Al-Hadidy, A, Masri, A, Hamamy, H, Reversade, B	Am J Hum Genet	2010
12907687	Chondroitin sulfate synthase-3. Molecular cloning and characterization Yada, T, Sato, T, Kaseyama, H, Gotoh, M, Iwasaki, H, Kikuchi, N, Kwon, YD, Togayachi, A, Kudo, T, Watanabe, H, Narimatsu, H, Kimata, K	J Biol Chem	2003
23774590	Biosynthesis and function of chondroitin sulfate Mikami, T, Kitagawa, H	Biochim Biophys Acta	2013