Pathophysiology of myasthenia gravis with antibodies to the acetylcholine receptor, muscle-specific kinase and low-density lipoprotein receptor-related protein 4

Jan J. G. M. Verschuuren*, Maartje G. Huijbers, Jaap J. Plomp, Erik H. Niks, Peter C. Molenaar, Pilar Martinez-Martinez, Alejandro M. Gomez, Marc H. De Baets, Mario Losen

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Myasthenia gravis is caused by antibodies to the acetylcholine receptor, muscle-specific kinase, low-density lipoprotein receptor-related protein 4, or possibly yet unidentified antibodies. The mechanisms by which these antibodies interfere with the function of postsynaptic proteins include complement activation, antigenic modulation by crosslinking of the target proteins, competition with ligand binding sites, or steric hindrance which inhibits conformational changes or binding to associated proteins. Screening for auto-antibodies to different postsynaptic targets, and also for low-affinity antibodies, is contributing to a more accurate diagnosis of MG patients. Further studies into the specific pathophysiological pathways of the several MG subforms might help to develop new, more antigen specific, therapies.
Original languageEnglish
Pages (from-to)918-923
JournalAutoimmunity Reviews
Volume12
Issue number9
DOIs
Publication statusPublished - Jul 2013

Keywords

  • Myasthenia gravis
  • Neuromuscular junction
  • MuSK
  • AChR
  • Lrp4

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