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

dc.contributor.author Yi, JS
dc.contributor.author Guidon, A
dc.contributor.author Sparks, S
dc.contributor.author Osborne, R
dc.contributor.author Juel, VC
dc.contributor.author Massey, JM
dc.contributor.author Sanders, DB
dc.contributor.author Weinhold, KJ
dc.contributor.author Guptill, JT
dc.coverage.spatial England
dc.date.accessioned 2015-06-16T19:33:36Z
dc.date.issued 2014-08
dc.identifier http://www.ncbi.nlm.nih.gov/pubmed/24378287
dc.identifier S0896-8411(13)00152-2
dc.identifier.uri https://hdl.handle.net/10161/10224
dc.description.abstract 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.
dc.language eng
dc.publisher Elsevier BV
dc.relation.ispartof J Autoimmun
dc.relation.isversionof 10.1016/j.jaut.2013.12.005
dc.subject Autoimmunity
dc.subject Human
dc.subject MuSK protein
dc.subject Myasthenia gravis
dc.subject Regulatory
dc.subject T-lymphocytes
dc.subject Adult
dc.subject Aged
dc.subject CD8-Positive T-Lymphocytes
dc.subject Cell Separation
dc.subject Cytokines
dc.subject Female
dc.subject Flow Cytometry
dc.subject Humans
dc.subject Immunoglobulin G
dc.subject Immunophenotyping
dc.subject Middle Aged
dc.subject Myasthenia Gravis
dc.subject Receptor Protein-Tyrosine Kinases
dc.subject Receptors, Cholinergic
dc.subject Sex Factors
dc.subject Th1 Cells
dc.subject Th17 Cells
dc.subject Young Adult
dc.title Characterization of CD4 and CD8 T cell responses in MuSK myasthenia gravis.
dc.type Journal article
duke.contributor.id Yi, JS|0549750
duke.contributor.id Juel, VC|0169399
duke.contributor.id Massey, JM|0114600
duke.contributor.id Sanders, DB|0111892
duke.contributor.id Guptill, JT|0400442
pubs.author-url http://www.ncbi.nlm.nih.gov/pubmed/24378287
pubs.begin-page 130
pubs.end-page 138
pubs.organisational-group Basic Science Departments
pubs.organisational-group Clinical Science Departments
pubs.organisational-group Duke
pubs.organisational-group Duke Cancer Institute
pubs.organisational-group Duke Clinical Research Institute
pubs.organisational-group Duke Human Vaccine Institute
pubs.organisational-group Immunology
pubs.organisational-group Institutes and Centers
pubs.organisational-group Neurology
pubs.organisational-group Neurology, Neuromuscular Disease
pubs.organisational-group Pathology
pubs.organisational-group School of Medicine
pubs.organisational-group Surgery
pubs.organisational-group Surgery, Surgical Sciences
pubs.publication-status Published
pubs.volume 52
dc.identifier.eissn 1095-9157
duke.contributor.orcid Yi, JS|0000-0001-7777-2437


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