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Therefore, specific neuronal roles have been attributed to this family of sialylated glycosphingolipids, e.g. in modulation of ion channels and transporters, neuronal interaction and recognition, temperature adaptation, Ca<jats:sup>2+<\/jats:sup>homeostasis, axonal growth, (para)node of Ranvier stability and synaptic transmission. Recent developmental, ageing and injury studies on transgenic mice lacking subsets of gangliosides indicate that gangliosides are involved in maintenance rather than development of the nervous system and that ganglioside family members are able to act in a mutually compensatory manner. Besides having physiological functions, gangliosides are the likely antigenic targets of autoantibodies present in Guillain\u2010Barr\u00e9 syndrome (GBS), a group of neuropathies with clinical symptoms of motor\u2010 and\/or sensory peripheral nerve dysfunction. Antibody binding to peripheral nerves is thought to either interfere with ganglioside function or activate complement, causing axonal damage and thereby disturbed action potential conduction. The presynaptic motor nerve terminal at the neuromuscular junction (NMJ) may be a prominent target because it is highly enriched in gangliosides and lies outside the blood\u2013nerve barrier, allowing antibody access. The ensuing neuromuscular synaptopathy might contribute to the muscle weakness in GBS patients. Several groups, including our own, have studied the effects of anti\u2010ganglioside antibodies in<jats:italic>ex vivo<\/jats:italic>and<jats:italic>in vivo<\/jats:italic>experimental settings at mouse NMJs. Here, after providing a background overview on ganglioside synthesis, localization and physiology, we will review those studies, which clearly show that anti\u2010ganglioside antibodies are capable of binding to NMJs and thereby can exert a variety of pathophysiological effects. Furthermore, we will discuss the human clinical electrophysiological and histological evidence produced so far of the existence of a neuromuscular synaptopathy contributing to muscle weakness in GBS patients.<\/jats:p>","DOI":"10.1113\/jphysiol.2009.171702","type":"journal-article","created":{"date-parts":[[2009,6,30]],"date-time":"2009-06-30T00:59:48Z","timestamp":1246323588000},"page":"3979-3999","source":"Crossref","is-referenced-by-count":81,"title":["Pathophysiological actions of neuropathy\u2010related anti\u2010ganglioside antibodies at the neuromuscular junction"],"prefix":"10.1113","volume":"587","author":[{"given":"Jaap J.","family":"Plomp","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugh 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