ganglioside biosynthetic process via lactosylceramide

Definition

Target type: biologicalprocess

The chemical reactions and pathways resulting in the formation of gangliosides that begins with the formation of lactosylceramides, Gal-beta-(1->4)-Glc-beta-(1->1') ceramides, any compound formed by the replacement of the glycosidic C1 hydroxyl group of lactose by a ceramide group. [GOC:bf, GOC:dph, GOC:tb]

The ganglioside biosynthetic process via lactosylceramide is a complex pathway that involves the sequential addition of sugar residues to a ceramide backbone. This process begins with the formation of lactosylceramide (LacCer), a glycosphingolipid that serves as the precursor for all gangliosides. LacCer is synthesized by the enzyme UDP-galactose:ceramide galactosyltransferase (CGT), which transfers galactose from UDP-galactose to ceramide. The resulting LacCer molecule then serves as a substrate for a series of glycosyltransferases that add additional sugar residues, including N-acetylgalactosamine (GalNAc), galactose (Gal), and sialic acid (Sia). The specific sequence of sugar additions determines the final structure of the ganglioside. For example, the addition of GalNAc to LacCer results in the formation of GM3, the simplest ganglioside. Subsequent addition of Gal to GM3 produces GM2, and further addition of Sia to GM2 results in GM1. These gangliosides are then further modified by the addition of additional sugar residues, such as Gal, GalNAc, and Sia, to form more complex gangliosides. Gangliosides are found in the outer leaflet of the plasma membrane and play a variety of important roles in cellular function, including cell adhesion, signal transduction, and neuronal development. They are also involved in various diseases, such as Tay-Sachs disease and Sandhoff disease, which are caused by defects in the enzymes that degrade gangliosides.'
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Proteins (1)

Compounds (11)