Pathophysiological actions of neuropathy-related anti-ganglioside antibodies at the neuromuscular junction.

摘要: The outer leaflet of neuronal membranes is highly enriched in gangliosides. Therefore, specific roles have been attributed to this family sialylated glycosphingolipids, e.g. modulation ion channels and transporters, interaction recognition, temperature adaptation, Ca2+ homeostasis, axonal growth, (para)node Ranvier stability synaptic transmission. Recent developmental, ageing injury studies on transgenic mice lacking subsets gangliosides indicate that are involved maintenance rather than development the nervous system ganglioside members able act a mutually compensatory manner. Besides having physiological functions, likely antigenic targets autoantibodies present Guillain-Barre syndrome (GBS), group neuropathies with clinical symptoms motor- and/or sensory peripheral nerve dysfunction. Antibody binding nerves thought either interfere function or activate complement, causing damage thereby disturbed action potential conduction. presynaptic motor terminal at neuromuscular junction (NMJ) may be prominent target because it lies outside blood–nerve barrier, allowing antibody access. ensuing synaptopathy might contribute muscle weakness GBS patients. Several groups, including our own, studied effects anti-ganglioside antibodies ex vivo experimental settings mouse NMJs. Here, after providing background overview synthesis, localization physiology, we will review those studies, which clearly show capable NMJs can exert variety pathophysiological effects. Furthermore, discuss human electrophysiological histological evidence produced so far existence contributing