IgG from patients with Guillain-Barré syndrome interact with nicotinic acetylcholine receptor channels

Muscle Nerve. 2003 Apr;27(4):435-41. doi: 10.1002/mus.10349.

Abstract

In Guillain-Barré syndrome (GBS), immunoglobulin G (IgG) antibodies block neuromuscular transmission pre- and postsynaptically and thus are of potential pathogenic relevance. We investigated whether IgG from GBS patients has a direct interaction with nicotinic acetylcholine receptor (nAChR) channels. Purified IgG fractions from six GBS patients that blocked neuromuscular transmission in a previous study were analyzed by the patch-clamp technique in combination with an ultrafast system for solution exchange. Sera from three patients with other inflammatory neurological disorders were used as controls. Mouse myotubes expressing native embryonic-type nAChR channels and human embryonic kidney (HEK) 293 cells transiently transfected with recombinant adult-type nAChR channels were used. Repeated 20-ms pulses of acetylcholine (ACh) were applied to outside-out patches in the presence of GBS-IgG. IgG of the patients had a significant reversible blocking action on embryonic- and adult-type nAChR channels with some variability in the magnitude of the block. Activation and desensitization kinetics were not affected when GBS-IgG was applied. None of the control sera blocked the AChR channels. The observed postsynaptic block effect fulfills the criteria of a channel-blocking IgG antibody similar to those seen in autoimmune myasthenia and may contribute to muscle weakness during the acute phase of GBS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Membrane / drug effects
  • Cell Membrane / immunology
  • Cell Membrane / metabolism
  • Cells, Cultured
  • Cholinergic Antagonists / immunology
  • Cholinergic Antagonists / pharmacology
  • Guillain-Barre Syndrome / immunology*
  • Guillain-Barre Syndrome / physiopathology
  • Humans
  • Immunoglobulin G / immunology*
  • Immunoglobulin G / pharmacology
  • Membrane Potentials / drug effects
  • Membrane Potentials / immunology
  • Mice
  • Muscle Weakness / immunology
  • Muscle Weakness / physiopathology
  • Muscle, Skeletal / physiopathology*
  • Neuromuscular Junction / immunology*
  • Neuromuscular Junction / physiopathology
  • Receptors, Nicotinic / drug effects
  • Receptors, Nicotinic / genetics
  • Receptors, Nicotinic / immunology*
  • Synaptic Transmission / drug effects
  • Synaptic Transmission / immunology*

Substances

  • Cholinergic Antagonists
  • Immunoglobulin G
  • Receptors, Nicotinic