Reactome | ST3GAL2,3 transfer Neu5Ac to gangliosides

ST3GAL2,3 transfer Neu5Ac to gangliosides

Stable Identifier

R-HSA-9845538

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

ReviewStatus

5/5

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ST3GAL2,3 transfer Neu5Ac to gangliosides

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The alpha-2,3-sialyltransferases 2 and 3 (ST3GAL2,3) transfer a sialic acid (Neu5Ac) moiety to the terminal galactosyl (Gal-R) residue of gangliosides GM1, GA1, and GD1b to produce GD1a, GM1b, and GT1a. Neu5Ac is added to Gal-R via an alpha-2,3 linkage (Kim et al., 1996; Grahn et al., 2002; Sturgill et al., 2012). Gangliosides localize to the plasma membrane via vesicle transport, endocytosis, and exocytosis. Defects in ST3GAL3 cause an intellectual developmental disorder (MRT12, MIM:611090; reviewed by Khamirani et al., 2021).

Literature References

PubMed ID	Title	Journal	Year
12815231	Cloning and sequencing of nineteen transcript isoforms of the human alpha2,3-sialyltransferase gene, ST3Gal III; its genomic organisation and expression in human tissues Grahn, A, Barkhordar, GS, Larson, G	Glycoconj J	2002
8920913	Molecular cloning and expression of human Gal beta 1,3GalNAc alpha 2,3-sialytransferase (hST3Gal II) Kim, YJ, Kim, KS, Kim, SH, Kim, CH, Ko, JH, Choe, IS, Tsuji, S, Lee, YC	Biochem Biophys Res Commun	1996
22735313	Biosynthesis of the major brain gangliosides GD1a and GT1b Sturgill, ER, Aoki, K, Lopez, PH, Colacurcio, D, Vajn, K, Lorenzini, I, Majić, S, Yang, WH, Heffer, M, Tiemeyer, M, Marth, JD, Schnaar, RL	Glycobiology	2012
34022416	Phenotype of ST3GAL3 deficient patients: A case and review of the literature Khamirani, HJ, Zoghi, S, Faghihi, F, Dastgheib, SA, Hassanipour, H, Bagher Tabei, SM, Mohammadi, S, Masoudi, M, Poorang, S, Ehsani, E, Dianatpour, M	Eur J Med Genet	2021