Animal Models of Myasthenia Gravis for Preclinical Evaluation

  • Linda L. Kusner
  • Rozen Le Panse
  • Mario Losen
  • William D. Phillips
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Myasthenia gravis (MG) is an autoimmune disorder characterized by generalized muscle weakness due to autoantibodies causing dysfunction of the neuromuscular junction (NMJ). Steroids and other immunosuppressant’s provide effective treatment for MG. However, these treatments often cause severe side effects, and some patients are treatment resistant. Therefore, the development of new therapeutics that are specific, potent, and safe is necessary to improve quality of life in MG patients. Use of animal models can accelerate the preclinical evaluation of new or repurposed therapeutics for MG. The experimental animal models are the experimental autoimmune MG (EAMG), induced by antigen immunization, and the passive transfer MG (PTMG), induced by antibodies. The variability, between laboratories, in experimental design, methods of induction, and outcome measurements for preclinical studies make it difficult to interpret and compare the published reports and assess the potential for application to MG patients. Standard procedures for animal care, sampling and randomization of animals, experimental design, and outcome measures will clarify the interpretation of preclinical testing and improve our ability to navigate potential new therapeutics toward clinical trials.

Keywords

Experimental autoimmune myasthenia gravis Passive transfer myasthenia gravis Experimental humanized mouse model Acetylcholine receptor myasthenia gravis models Muscle-specific kinase myasthenia gravis models Outcome measurements Electromyography Animal care Rigor 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Linda L. Kusner
    • 1
  • Rozen Le Panse
    • 2
  • Mario Losen
    • 3
  • William D. Phillips
    • 4
  1. 1.Department of Pharmacology and PhysiologyGeorge Washington UniversityWashington, DCUSA
  2. 2.UMRS 974 – UPMC Sorbonne Universités – INSERM – AIMParisFrance
  3. 3.Department of NeuroscienceSchool of Mental Health and Neuroscience, Maastricht UniversityMaastrichtThe Netherlands
  4. 4.School of Medical Sciences (Physiology) and Bosch Institute, Anderson Stuart Bldg (F13)University of SydneySydneyAustralia

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