| Modification Notes |
GM3 Synthase, also known as Sialyltransferase 9 or ST3Gal V, is a Golgi type II transmembrane glycosyltransferase predominantly expressed in brain and placenta. It belongs to the glycosyltransferase family 29 and is involved in the biosynthesis of complex gangliosides. In particular, GM3 Synthase catalyzes the transfer of å sialic acid to the terminal galactose of lactosylceramide to form the ganglioside GM3. GM3 is the simplest ganglioside and it participates in cell differentiation, signal transduction, and modulation of cell proliferation. The synthesis of GM3 by GM3 Synthase is the first major step in the formation of almost all other gangliosides. For this reason, GM3 Synthase acts as a key regulatory enzyme in the biosynthesis of gangliosides. A mutation in the gene encoding GM3 Synthase can lead to the inability to synthesize å- and ∫-series gangliosides and may result in Amish infantile epilepsy syndrome. |