Ganglioside mimicry as a cause of Guillain-Barré syndrome.

摘要: Guillain-Barre syndrome (GBS), characterized by acute progressive limb weakness and areflexia, is the prototype of postinfectious autoimmune diseases. Campylobacter jejuni most frequently identified agent infection in GBS patients, often preceding motor axonal neuropathy (AMAN), a variant GBS. Anti-GM1, anti-GM1b, anti-GD1a, anti-GalNAc-GD1a IgG antibodies are associated with AMAN. Carbohydrate mimicry [Galbeta1-3GalNAcbeta1-4(NeuAcalpha2-3)Galbeta1-] was seen between lipo-oligosaccharide C. isolated from an AMAN patient human GM1 ganglioside. Sensitization induces rabbits as does sensitization Paralyzed have pathological changes their peripheral nerves identical to may induce anti-ganglioside molecular mimicry, eliciting This first verification causative mechanism disease. To express ganglioside mimics, requires specific gene combinations that function sialic acid biosynthesis or transfer. The knockout mutants these landmark genes show reduced reactivity patients' sera, fail antibody response mice. These crucial for induction neuropathogenic cross-reactive antibodies. An approach evaluating intravenous immune globulin, treatment GBS, based on our animal model also discussed this review, recent advances made field described.