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. 2012 Dec 11;88(10):554–582. doi: 10.2183/pjab.88.554

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© 2012 The Japan Academy

This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Figure 9. — Scheme of ganglioside biosynthesis. Major gangliosides occurring on neurons in adult mammalian brain are underlined. Enzyme defects in knockout mice are indicated. A, ceramide glucosyltransferase −/−; B, galactosyltransferase I −/−; C, sialyltransferase I −/−; D, sialyltransferase II −/−; E, GalNAc-transferase −/−; “GM3 only” mouse = D + E; “LacCer only” mouse = C + E. GD1α, GT1α, GQ1bα and GP1cα with a sialic acid residue α2,6-glycosidically linked to N-acetylgalactosamine are formed by a yet uncharacterized sialyltransferase, whereas GD1c, GT1a, GQ1b and GP1c are formed by SAT V T (modified after ref. 211).