Reactome | ST3GAL5 transfers NeuNAc to ceramides

ST3GAL5 transfers NeuNAc to ceramides

Stable Identifier

R-HSA-9844860

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

ReviewStatus

5/5

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Following its biosynthesis, a fraction of GalCer reaches the lumen of the Golgi and is reacted with cytidine-5′-monophospho-N-acetylneuraminic acid by the action of the sialyltransferase ST3GAL5 to form N-acetylneuraminyl-GalCer (GM4; sialyl-GalCer) (Shanker and Pieringer, 1983; Ishii et al., 1998; reviewed by Liu & Li, 2013). ST3GAL5 also catalyzes the sialylation of lactosylceramide (LacCer) to ganglioside GM3 and the sialylation of gangliosides GA1/GA2 to gangliosides GM1/GM2. The process is localized in the Golgi membrane (Allende et al., 2000; Stern et al., 2000; Berselli et al., 2006). Deficiencies in ST3GAL5 are the cause of "Salt and Pepper" developmental regression syndrome (SPDRS, MIM:609056; Aoki et al., 2019; reviewed by Inamori & Inokuchi, 2022).

Literature References

PubMed ID	Title	Journal	Year
11030748	Evidence supporting a late Golgi location for lactosylceramide to ganglioside GM3 conversion Darling, DS, Li, J, Allende, ML, Young, WW, Worth, CA	Glycobiology	2000
35628171	Ganglioside GM3 Synthase Deficiency in Mouse Models and Human Patients Inokuchi, JI, Inamori, KI	Int J Mol Sci	2022
23290777	Ceramide glycosylation catalyzed by glucosylceramide synthase and cancer drug resistance Liu, YY, Hill, RA, Li, YT	Adv. Cancer Res.	2013
34917767	Mass spectrometric quantification of plasma glycosphingolipids in human GM3 ganglioside deficiency Aoki, K, Tiemeyer, M, Strauss, KA, Heaps, AD	Clin Mass Spectrom	2019
9822625	Expression cloning and functional characterization of human cDNA for ganglioside GM3 synthase Matsuda, K, Saito, M, Inokuchi, J, Ishii, A, Sakoe, K, Sanai, Y, Ohta, M, Ishiyama, K, Watanabe, Y, Nakamura, M	J. Biol. Chem.	1998
16934889	Human GM3 synthase: a new mRNA variant encodes an NH2-terminal extended form of the protein Sottocornola, E, Colombo, I, Berra, B, Milani, S, Zava, S, Berselli, P	Biochim Biophys Acta	2006
10764824	Molecular identification, tissue distribution and subcellular localization of mST3GalV/GM3 synthase Braverman, TR, Stern, CA, Tiemeyer, M	Glycobiology	2000