Animal models of antimuscle-specific kinase myasthenia

David P Richman, Kayoko Nishi, Michael J Ferns, Joachim Schnier, Peter Pytel, Ricardo A Maselli, Mark A. Agius

Research output: Contribution to journalArticle

6 Scopus citations

Abstract

Antimuscle-specific kinase (anti-MuSK) myasthenia (AMM) differs from antiacetylcholine receptor myasthenia gravis in exhibiting more focal muscle involvement (neck, shoulder, facial, and bulbar muscles) with wasting of the involved, primarily axial, muscles. AMM is not associated with thymic hyperplasia and responds poorly to anticholinesterase treatment. Animal models of AMM have been induced in rabbits, mice, and rats by immunization with purified xenogeneic MuSK ectodomain, and by passive transfer of large quantities of purified serum IgG from AMM patients into mice. The models have confirmed the pathogenic role of the MuSK antibodies in AMM and have demonstrated the involvement of both the presynaptic and postsynaptic components of the neuromuscular junction. The observations in this human disease and its animal models demonstrate the role of MuSK not only in the formation of this synapse but also in its maintenance.

Original languageEnglish (US)
Pages (from-to)140-147
Number of pages8
JournalAnnals of the New York Academy of Sciences
Volume1274
Issue number1
DOIs
StatePublished - Dec 2012

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Keywords

  • Animal models
  • Autoimmune
  • Muscle wasting
  • Muscle-specific kinase
  • MuSK
  • Myasthenia
  • Neuromuscular junction
  • Synapse

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • History and Philosophy of Science

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