Novel CXCL13 transgenic mouse: Inflammation drives pathogenic effect of CXCL13 in experimental myasthenia gravis

Julia Miriam Weiss, Marieke Robinet, Revital Aricha, Perrine Cufi, Bérengère Villeret, Frida Lantner, Idit Shachar, Sara Fuchs, Miriam C. Souroujon, Sonia Berrih-Aknin, Rozen Le Panse

Research output: Contribution to journalArticlepeer-review


Abnormal overexpression of CXCL13 is observed in many inflamed tissues and in particular in autoimmune diseases. Myasthenia gravis (MG) is a neuromuscular disease mainly mediated by anti-acetylcholine receptor autoantibodies. Thymic hyperplasia characterized by ectopic germinal centers (GCs) is a common feature in MG and is correlated with high levels of anti-AChR antibodies. We previously showed that the B-cell chemoattractant, CXCL13 is overexpressed by thymic epithelial cells in MG patients. We hypothesized that abnormal CXCL13 expression by the thymic epithelium triggered B-cell recruitment in MG. We therefore created a novel transgenic (Tg) mouse with a keratin 5 driven CXCL13 expression. The thymus of Tg mice overexpressed CXCL13 but did not trigger B-cell recruitment. However, in inflammatory conditions, induced by Poly(I:C), B cells strongly migrated to the thymus. Tg mice were also more susceptible to experimental autoimmune MG (EAMG) with stronger clinical signs, higher titers of anti-AChR antibodies, increased thymic B cells, and the development of germinal center-like structures. Consequently, this mouse model finally mimics the thymic pathology observed in human MG. Our data also demonstrated that inflammation is mandatory to reveal CXCL13 ability to recruit B cells and to induce tertiary lymphoid organ development.

Original languageEnglish
Pages (from-to)7550-7562
Number of pages13
Issue number7
StatePublished - 2016

Bibliographical note

Funding Information:
This work was supported by grants from the "Agence Nationale de la Recherche" (ANR-06-MRAR-001-01), from the European Community (MYASTAID/LSHM-CT-2006-037833 and FIGHT-MG/HEALTH-2009-242-210) and from the "Association Fran?aise contre les Myopathies" (AFM).


  • Autoimmunity
  • B cells
  • CXCL13-CXCR5
  • Chemokine
  • Immune response
  • Immunity
  • Immunology and microbiology section
  • Thymus


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