Ganglioside GM1 is a monosialo-glycosphingolipid belonging to the gangliotetrahexosyl series that abundant in neurons of all animal species and plays important roles in many cell physiological processes, including differentiation, memory control, cell signaling, neuronal protection, neuronal recovery, and apoptosis. Ganglioside GM1 in neurons helps to transfer information from the exterior to the interior of the cell, through specific recognition and binding of biologically active molecules (membrane receptors and ion channels), and has specific functions in nerve conduction and/or synaptic transmission. The mechanisms underlying the effects of Ganglioside GM1 remain unclear in many cases, but it appears that these effects are often due to specific interactions between Ganglioside GM1 and proteins involved in signaling processes, within Ganglioside GM1-enriched lipid rafts in the plasma membrane. Ganglioside GM1 is a major component of total ganglioside mixtures from mammalian brains, from which it can be extracted and purified in large amounts. Ganglioside GM1 was widely used in the past as a therapeutic drug for a wide variety of neurological disorders. Further studies have shown that Ganglioside GM1 has immunogenic properties and led to the production of antibodies that promoted peripheral neuropathies such as Guillain–Barré syndrome.