Reactome | CSGALNACTs transfer GalNAc to CS linker glycan

CSGALNACTs transfer GalNAc to CS linker glycan

Stable Identifier

R-HSA-1971482

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

Synonyms

The addition of GalNAc to the terminal glucuronate residue forms chondroitin

ReviewStatus

5/5

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CSGALNACTs transfer GalNAc to CS linker glycan

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Chondroitin sulfate N-acetylgalactosaminyltransferases 1 and 2 (CSGALNACT1 and 2) (Uyama et al. 2002, Gotoh et al. 2002) transfer N-acetylgalactosamine (GalNAc) from UDP-GalNAc to the glucuronate (GlcA) residue of the linker sequence. This first addition to the linker determines this GAG to be chondroitin. Chondroitin is comprised of the repeating disaccharide unit GalNAc-GlcA. Mutations in CSGALNACT1 can lead to mild skeletal dysplasia (SDJLABA, MIM:618870; Vodopiutz et al., 2017).

Literature References

PubMed ID	Title	Journal	Year
11788602	Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfate Uyama, T, Kitagawa, H, Tamura Ji, J, Sugahara, K	J Biol Chem	2002
12163485	Enzymatic synthesis of chondroitin with a novel chondroitin sulfate N-acetylgalactosaminyltransferase that transfers N-acetylgalactosamine to glucuronic acid in initiation and elongation of chondroitin sulfate synthesis Gotoh, M, Sato, T, Akashima, T, Iwasaki, H, Kameyama, A, Mochizuki, H, Yada, T, Inaba, N, Zhang, Y, Kikuchi, N, Kwon, YD, Togayachi, A, Kudo, T, Nishihara, S, Watanabe, H, Kimata, K, Narimatsu, H	J Biol Chem	2002
27599773	Chondroitin Sulfate N-acetylgalactosaminyltransferase-1 (CSGalNAcT-1) Deficiency Results in a Mild Skeletal Dysplasia and Joint Laxity Vodopiutz, J, Mizumoto, S, Lausch, E, Rossi, A, Unger, S, Janocha, N, Costantini, R, Seidl, R, Greber-Platzer, S, Yamada, S, Müller, T, Jilma, B, Ganger, R, Superti-Furga, A, Ikegawa, S, Sugahara, K, Janecke, AR	Hum Mutat	2017