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Immune responses to myelin proteins in Guillain-Barré syndrome
  1. Anna Makowska (popywhite60{at}hotmail.com)
  1. Departments of Clinical Neuroscience & Immunobiology, King's College London, United Kingdom
    1. Jane Pritchard (jpritchard{at}doctors.org.uk)
    1. Departments of Clinical Neuroscience and Immunobiology, King's College London, United Kingdom
      1. Lara Sanvito (lara.sanvito{at}kcl.ac.uk)
      1. Department of Clinical Neuroscience, King's College London, United Kingdom
        1. Norman Gregson (norman.gregson{at}kcl.ac.uk)
        1. Department of Clinical Neuroscience, King's College London, United Kingdom
          1. Mark Peakman (mark.peakman{at}kcl.ac.uk)
          1. Department of Immunobiology, King's College London, United Kingdom
            1. Adrian Hayday (adrian.hayday{at}kcl.ac.uk)
            1. Department of Immunobiology, King's College London, Uzbekistan
              1. Richard Hughes (richard.a.hughes{at}kcl.ac.uk)
              1. Department of Clinical Neuroscience, King's College London, United Kingdom

                Abstract

                Background: Potential target autoantigens in the demyelinating form of Guillain-Barré Syndrome (GBS) include the myelin proteins PMP22, P0 and P2.

                Methods: We investigated immunoreactivity to P0, P2 and PMP22 proteins in 37 GBS patients and 32 healthy controls.

                Results: Antibodies to PMP22 or P0 peptides were detected at presentation in only 5 out of 37 patients. In ELISPOT assays, blood mononuclear cells from 15 out of 24 GBS patients but none of the control subjects produced interleukin-10 (IL-10) in response to peptides from proteins P0, P2 or PMP22 (p = 0.0003). The cells from only two patients produced interferonγ (IFNγ).The cells from 11 GBS patients had increased IL-10 responses to peptides representing sequences from the extracellular domains of PMP22 before intravenous immunoglobulin (IVIg) treatment (p = 0.006). The cells from 11 GBS patients, including seven who responded to the extracellular domains of PMP22, had increased IL-10 responses to the intracellular domain of P0 before (p = 0.005) and those from 9 patients after they had been treated with IVIg (p = 0.01).

                Conclusions: Antibodies to P0 and PMP22 protein peptides do occur in GBS but are uncommon. Circulating mononuclear cell IFNγ responses to P0 and PMP22 myelin proteins are rare but IL-10 responses occur significantly more often than in normal subjects. They might be part of a harmful pathogenetic process or represent a regulatory response.

                • Myelin
                • Guillain-Barré syndrome
                • antibody
                • T cell
                • ELISPOT

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