= aNeu5Ac(2-3)bDGalp(1-1)Cer where aNeu5Ac = N-acetyl-alpha-neuraminic acid aNeu5Ac9Ac = N-acetyl-9-O-acetylneuraminic acid bDGalp = beta-D-galactopyranose bDGalpNAc = N-acetyl-beta-D-galactopyranose bDGlcp = beta-D-glucopyranose Cer = ceramide (general N-acylated sphingoid) Pathology Gangliosides are continuously synthesized and degraded in cells. They are degraded to ceramides by sequential removal of sugar units in the oligosaccharide group, catalyzed by a set of highly specific lysosomal enzymes. Mutations in genes coding for these enzymes leads to the accumulation of partially broken down gangliosides in lysosomes, which results in a group of diseases called gangliosidosis. For example, the fatal Tay–Sachs disease arises as a genetic defect which leads to no functional hexosaminidase A produced, causing GM2 to accumulate in lysosomes. Ultimately the ganglion cells in the nervous system swell enormously, disturbing the normal functions of neurons. Gangliosides are also involved in several diseases: Influenza, in which haemagglutinin of influenza virus exploits certain gangliosides to enter and infect the cells expressing them. Guillain–Barré syndrome, which has been linked to the production of anti-ganglioside antibodies. Cholera Tetanus Botulism Leprosy Obesity, where inadequate ganglioside expression in mediobasal hypothalamic neurons deregulates neuronal leptin and insulin signaling. References External links Overview of gangliosides at lipidlibrary.co.uk Overview of gangliosides at cyberlipid.org Glycolipids