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J Neurochem. 2011 Mar;116(5):828-32. doi: 10.1111/j.1471-4159.2010.07029.x. Epub 2011 Jan 7.

Antibodies against ganglioside complexes in Guillain-Barré syndrome and related disorders.

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1
Department of Neurology, Kinki University School of Medicine, Osaka, Japan. kusunoki-tky@umin.ac.jp

Abstract

Guillain-Barré syndrome (GBS) is acute autoimmune neuropathy, often subsequent to an infection. Serum anti-ganglioside antibodies are frequently elevated in titer. Those antibodies are useful diagnostic markers and possible pathogenetic factors. Recent data demonstrated that sera from some patients with GBS react with ganglioside complexes (GSCs) consisting of two different gangliosides, but not with each constituent ganglioside. Those antibodies may specifically recognize a new conformational epitope formed by two gangliosides. In particular, the antibodies against GD1a/GD1b and/or GD1b/GT1b complexes are associated with severe GBS requiring artificial ventilation. The antibodies to GM1/GalNAc-GD1a and those to GSCs containing GQ1b or GT1a are associated with pure motor GBS and Fisher syndrome, respectively. In contrast, the binding activities of the antibodies highly specific to GD1b are strongly inhibited by the addition of GD1a to GD1b. Gangliosides along with other components as cholesterol are known to form lipid rafts, in which two different gangliosides may form a new conformational epitope. Future investigation is necessary to elucidate the roles of GSCs in the plasma membrane and of the clinical relevance of the anti-GSCs antibodies.

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