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Defects of immunoregulatory mechanisms in myasthenia gravis: role of IL‐17
Author(s) -
Gradolatto Angeline,
Nazzal Dani,
Foti Maria,
Bismuth Jacky,
Truffault Frederique,
Panse Rozen Le,
BerrihAknin Sonia
Publication year - 2012
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.2012.06791.x
Subject(s) - myasthenia gravis , immunology , t cell , il 2 receptor , interleukin 7 receptor , inflammation , biology , downregulation and upregulation , regulatory t cell , immune system , gene , genetics
Deficient immunoregulation is consistently observed in autoimmune diseases. Here, we summarize the abnormalities of the T cell response in autoimmune myasthenia gravis (MG) by focusing on activation markers, inflammatory features, and imbalance between the different T cell subsets, including Th17 and regulatory T cells (T reg cells). In the thymus from MG patients, T reg cell numbers are normal while their suppressive function is severely defective, and this defect could not be explained by contaminating effector CD127 low T cells. A transcriptomic analysis of T reg cell and conventional T cell (T conv ; CD4 + CD25 − cells) subsets pointed out an upregulation of Th17‐related genes in MG cells. Together with our previous findings of an inflammatory signature in the MG thymus and an overproduction of IL‐1 and IL‐6 by MG thymic epithelial cells (TEC), these data strongly suggest that T cell functions are profoundly altered in the thymic pathological environment. In this short review we discuss the mechanisms of chronic inflammation linked to the pathophysiology of MG disease.