Characterization of CD4 and CD8 T cell responses in MuSK myasthenia gravis.


Muscle specific tyrosine kinase myasthenia gravis (MuSK MG) is a form of autoimmune MG that predominantly affects women and has unique clinical features, including prominent bulbar weakness, muscle atrophy, and excellent response to therapeutic plasma exchange. Patients with MuSK MG have predominantly IgG4 autoantibodies directed against MuSK on the postsynaptic muscle membrane. Lymphocyte functionality has not been reported in this condition. The goal of this study was to characterize T cell responses in patients with MuSK MG. Intracellular production of IFN-gamma, TNF-alpha, IL-2, IL-17, and IL-21 by CD4+ and CD8+ T cells was measured by polychromatic flow cytometry in peripheral blood samples from 11 Musk MG patients and 10 healthy controls. Only one MuSK MG patient was not receiving immunosuppressive therapy. Regulatory T cells (Treg) were also included in our analysis to determine if changes in T cell function were due to altered Treg frequencies. CD8+ T cells from MuSK MG patients had higher frequencies of polyfunctional responses than controls, and CD4+ T cells had higher IL-2, TNF-alpha, and IL-17. MuSK MG patients had a higher percentage of CD4+ T cells producing combinations of IFN-gamma/IL-2/TNF-gamma, TNF-alpha/IL-2, and IFN-gamma/TNF-alpha. Interestingly, Treg numbers and CD39 expression were not different from control values. MuSK MG patients had increased frequencies of Th1 and Th17 cytokines and were primed for polyfunctional proinflammatory responses that cannot be explained by a defect in CD39 expression or Treg number.





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Publication Info

Yi, JS, A Guidon, S Sparks, R Osborne, VC Juel, JM Massey, DB Sanders, KJ Weinhold, et al. (2014). Characterization of CD4 and CD8 T cell responses in MuSK myasthenia gravis. J Autoimmun, 52. pp. 130–138. 10.1016/j.jaut.2013.12.005 Retrieved from

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John S Yi

Adjunct Assistant Professor in the Department of Surgery

I am an immunologist, with a focus to characterize the immune system in response to infectious and non-infectious diseases including cancer, HIV, autoimmune disease, and transplantation. My goals are to identify novel biomarkers/immune signatures that clinicians can utilize to diagnosis, predict disease outcomes, and determine patients' response to treatment. 


Vern Charles Juel

Professor of Neurology

Janice Munn Massey

Professor of Neurology

Clinical Research in Neuromuscular diseases including myasthenia gravis, Lambert-Eaton myasthenic syndrome, botulinum toxins, electromyography, dystonic disorders including cervical dystonia (spasmodic torticollis), limb focal dystonia, and blepharospasm.


Donald Benjamin Sanders

Professor of Neurology

Research Interests:
1. Therapy of neuromuscular disease - immunologic and neuromuscular facilitating agents for myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS).
2. Clinical trials in nerve, muscle and neuromuscular diseases


Jeffrey Guptill

Adjunct Associate Professor in the Department of Neurology

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