CSGALNACTs transfer GalNAc to CS chain

Stable Identifier
R-HSA-9632033
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
GalNAc is added to chondroitin
ReviewStatus
5/5
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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 terminal glucuronate (GlcA) residue of the CS chain. CSGALNACTs may also regulate CS chain length (reviewed in Mikami & Kitagawa, 2013). 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
23774590 Biosynthesis and function of chondroitin sulfate

Mikami, T, Kitagawa, H

Biochim Biophys Acta 2013
Participants
Participates
Catalyst Activity

glucuronosyl-N-acetylgalactosaminyl-proteoglycan 4-beta-N-acetylgalactosaminyltransferase activity of CSGALNACT [Golgi membrane]

Orthologous Events
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