The auto-antigen repertoire in myasthenia gravis

Autoimmunity. 2010 Aug;43(5-6):380-400. doi: 10.3109/08916930903518073.

Abstract

Myasthenia Gravis (MG) is an antibody-mediated autoimmune disorder affecting the postsynaptic membrane of the neuromuscular junction (NMJ). MG is characterized by an impaired signal transmission between the motor neuron and the skeletal muscle cell, caused by auto-antibodies directed against NMJ proteins. The auto-antibodies target the nicotinic acetylcholine receptor (nAChR) in about 90% of MG patients. In approximately 5% of MG patients, the muscle specific kinase (MuSK) is the auto-antigen. In the remaining 5% of MG patients, however, antibodies against the nAChR or MuSK are not detectable (idiopathic MG, iMG). Although only the anti-nAChR and anti-MuSK auto-antibodies have been demonstrated to be pathogenic, several other antibodies recognizing self-antigens can also be found in MG patients. Various auto-antibodies associated with thymic abnormalities have been reported, as well as many non-MG-specific auto-antibodies. However, their contribution to the cause, pathology and severity of the disease is still poorly understood. Here, we comprehensively review the reported auto-antibodies in MG patients and discuss their role in the pathology of this autoimmune disease.

Publication types

  • Review

MeSH terms

  • Animals
  • Autoantibodies / blood
  • Autoantibodies / classification
  • Autoantibodies / immunology*
  • Autoantigens / immunology*
  • Autoimmune Diseases / immunology
  • Cytokines / immunology
  • Humans
  • Muscle Weakness / immunology
  • Myasthenia Gravis / immunology*
  • Myasthenia Gravis / physiopathology
  • Neuromuscular Junction / immunology
  • Neuromuscular Junction / physiopathology
  • Receptor Protein-Tyrosine Kinases / immunology*
  • Receptors, Cholinergic / immunology*
  • Receptors, Nicotinic / immunology*
  • Thymus Gland / immunology
  • Thymus Gland / physiopathology

Substances

  • Autoantibodies
  • Autoantigens
  • Cytokines
  • Receptors, Cholinergic
  • Receptors, Nicotinic
  • MUSK protein, human
  • Receptor Protein-Tyrosine Kinases