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Highly Accessed Open Badges Review

Antigen receptor signaling in the rheumatic diseases

Julie Zikherman and Arthur Weiss*

Author Affiliations

Division of Rheumatology, Rosalind Russell Medical Research Center for Arthritis, Department of Medicine, Howard Hughes Medical Institute, University of California, San Francisco, 513 Parnassus Avenue San Francisco, CA 94143, USA

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Arthritis Research & Therapy 2009, 11:202  doi:10.1186/ar2528

Published: 30 January 2009


Antigen receptor signaling in lymphocytes has been clearly implicated in the pathogenesis of the rheumatic diseases. Here, we review evidence from mouse models in which B-cell and T-cell signaling machinery is perturbed as well as data from functional studies of primary human lymphocytes and recent advances in human genetics. B-cell receptor hyper-responsiveness is identified as a nearly universal characteristic of systemic lupus erythema-tosus in mice and humans. Impaired and enhanced T-cell receptor signaling are both associated with distinct inflammatory diseases in mice. Mechanisms by which these pathways contribute to disease in mouse models and patients are under active investigation.