Identification of a gene associated with Guillain-Barré syndrome (GBS) suggests pathogenesis of the disease. (IMAGE)
Caption
Patients with GBS characteristically produce autoantibodies to sialic acid-containing glycolipids called gangliosides present in neuronal cells. These autoantibodies are thought to bind to gangliosides present on the surface of neurons, and cause paralysis and sensory disturbance by damaging neurons. Here we demonstrate that a rare variant of the molecule called Siglec-10 accumulates in patients with GBS. Siglec-10 is a cell surface molecule present in various immune cells including antibody-producing cells. Siglec-10 recognizes sialic acid-containing molecules including gangliosides as ligands, and is known to inhibit activation of antibody-producing cells upon interaction with the ligands. Normal Siglec-10 may inhibit activation of anti-ganglioside antibody-producing cells when these cells interact with gangliosides present in neurons. In contrast, the Siglec-10 variant accumulated in patients with GBS shows impaired binding to gangliosides. Therefore, this Siglec-10 variant may fail to inhibit activation of anti-ganglioside antibody-producing cells, thereby allowing production of anti-ganglioside autoantibodies.
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Department of Immunology, TMDU
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