ganglioside

Gangliosides are a diverse class of glycosphingolipids that contain one or more sialic acid residues. They reside primarily on the outer leaflet of the plasma membrane and are especially abundant in the nervous system, where they contribute to membrane stability, cell–cell interaction, and signal transduction. Gangliosides are categorized by the number of sialic acids and are given nomenclatures such as GM, GD, and GT (for example GM1, GM2, GM3, GD1a, GD3).

Biosynthesis occurs in the Golgi apparatus from ceramide. Enzymes sequentially add sugar residues to form precursor glycosphingolipids, followed by sialylation to generate the various ganglioside series. After assembly, gangliosides are transported to the plasma membrane. Degradation takes place in lysosomes through specific hydrolases; defects in these enzymes can lead to lysosomal storage disorders, including Tay-Sachs disease (accumulation of GM2) and GM1 gangliosidosis (accumulation of GM1).

Functions of gangliosides include modulation of receptor activity and signal transduction, participation in neuronal development and synaptic plasticity, and involvement in cell–cell recognition. They are enriched in lipid rafts and can act as receptors or co-receptors for certain toxins and pathogens; notably, the B subunit of cholera toxin binds GM1. Autoimmune anti-ganglioside antibodies are associated with Guillain-Barré syndrome and related neuropathies.

Because of their prominence in nervous tissue, gangliosides are important in neuroscience and metabolic disease research, with ongoing study of their roles in development, aging, and neurodegeneration.