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. 2018 Nov 26;10(8):550–565. doi: 10.1007/s13238-018-0597-5

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© The Author(s) 2018

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

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The biosynthesis pathway of sialylation. The nucleotide sugar UDP-GlcNAc, the production of hexosamine pathway, is converted into ManNAc by UDP-GlcNAc 2-epimerase (whose encoding gene is GNE in human). ManNAc is metabolic precursor for the synthesis of sialic acid and produces Neu5Ac in the cytosol, which then enters the nucleus to produce CMP-Neu5Ac. CMP-Neu5Ac are transported into Golgi where they are used by ST3GAL1-6, ST6GAL1-2/ST6GALNAC1-6, ST8SIA4 to produce α-2,3-, α-2,6- and α-2,8-linked sialoglycoproteins or gangliosides, respectively. Finally, sialosides are recycled by neuraminidases, regenerating sialic acid monomers that can be re-used