Muscle-Specific Receptor Tyrosine Kinase (MuSK) Myasthenia Gravis

Curr Neurol Neurosci Rep. 2016 Jul;16(7):61. doi: 10.1007/s11910-016-0668-z.

Abstract

Autoimmune myasthenia gravis (MG) is the prototypic, antibody-mediated neuromuscular disease and is characterized by a decrease in the number of functional acetylcholine receptors (AChR) within the muscle end plate zone of the neuromuscular junction (NMJ). Although the pathophysiology of AChR-mediated myasthenia gravis has been extensively studied over the last 40 years since its original description by Patrick and Lindstrom (Science 180:871-872, 1973), less is known about the much more recently described muscle-specific kinase (MuSK) antibody-mediated MG. MuSK-MG has features clinically distinct from Ach-R MG, as well as a different pattern of response to treatment and a unique immunopathogenesis.

Keywords: Acetylcholine receptor antibody; Muscle-specific receptor tyrosine kinase; Myasthenia gravis; Neuromuscular junction.

Publication types

  • Review

MeSH terms

  • Autoantibodies / immunology*
  • Humans
  • Myasthenia Gravis* / diagnosis
  • Myasthenia Gravis* / immunology
  • Myasthenia Gravis* / therapy
  • Receptor Protein-Tyrosine Kinases / immunology*
  • Receptors, Cholinergic / immunology*

Substances

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